Wednesday (Blood and GI)
Scrofula 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Acute Nonspecific lymphadenitis 2. No 3. Cervical lymphadenitis due to mycobacterial species: adults (>90% TB), children (>90% atypical mycobacterium), immunosuppressed (atypical) 4. N/A 5. Painfully, enlarged nodes. May see neutrophilic infiltrates and abscess formation/necrosis. Boboes (yersinia pestis, gonorrhea, TB, atypcial mycobacteria, syphilis) 6. N/A 7. N/A
Acute Nonspecific Lymphadenitis General 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Acute Nonspecific lymphadenitis 2. No 3. Commonly seen with bacterial infections, some viral. Seen near focal infection: cervical (abscessed tooth/tonsillitis), axillary/inguinal (extremity infection), mesenteric (acute appendicitis). 4. Follicular hyperplasia with large germinal centers. 5. Painfully, enlarged nodes. May see neutrophilic infiltrates and abscess formation/necrosis. Boboes (yersinia pestis, gonorrhea, TB, atypcial mycobacteria, syphilis) 6. N/A 7. N/A
Chronic Blood Loss Anemia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Blood Loss 2. N/A 3. Slow blood loss in GI/GU 4. Microcytic 5. N/A 6. N/A 7. Iron Deficiency Anemia Sx 8. Fix Cause 9. N/A
Acute Blood Loss Anemia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Blood Loss 2. N/A 3. Trauma 4. Normal 5. Initially: Normal; - 3-5 Days after: reticulocytosis & polychromasia, leukocytosis & thrombocytosis 6. Normal 7. Increased HR, BP; decreased tissue perfusion 8. Saline 9. N/A
Sinus Histiocytosis 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Chronic Nonspecific Lymphadenitis 2. No 3. Distention of lymph nodes sinuses due to endothelial cell swelling and increased histiocytes (mphages). Common in nodes draining cancer. 4. N/A 5. N/A 6. N/A 7. Common in inguinal/axillary lymph nodes
Follicular Hyperplasia 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Chronic Nonspecific Lymphadenitis 2. No 3. Expansion of lymphoid follicles (B cell proliferation and ab production). Can be due to Rh. Arthritis, toxoplasmosis, ect. 4. Tingible body macrophages may be a sign of malignancy. 5. Non-tender lymphadenitis, no acute inflammation 6. N/A 7. Common in inguinal/axillary lymph nodes
Paracortical Hyperplasia 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Chronic Nonspecific Lymphadenitis 2. No 3. Reactive changes in T cell regions that efface/destroy normal follicular structure. Often due to viral infections (mono), post-vaccine (smallpox), drug reaction (dilantin), and lymphoma. 4. N/A 5. N/A 6. N/A 7. Common in inguinal/axillary lymph nodes
Autoimmune AKA AIHA: Warm. Drug Induced - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Autoantibodies (IgG) against self very common RBC antigens due to antigenic drugs (bind RBC and anti-drug Abs react [penicillin/cephalosporin]) or tolerance-breaking drugs ([a-methyldopa] drug induces abs against RBC) 4. Normal to Microcytic 5. Microspherocytes, Reticulocytosis/Polychromasia, schistocytes, nucleated RBCs 6. Elevated EPO, Bilirubin, LDH, and urine urobilinogen. Direct Coombs test+. - Rarely can have decreased haptoglobin with hemoglobinuria/hemoglobinemia. 7. Anemia jaundice/icterus,, gallstones unexplained fever, moderate splenomegaly. 8. Treat underlying disease. Avoid transfusions. Plasma exchange. Corticosteroids or Immunosuppressive. Splenectomy. 9. Patients serum agglutinates all reagent/donor cells. Optimal serum activity at 37C.
Hereditary Spherocytosis and Elliptocytosis - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. AD, defects in a membrane protein > unstable membrane removed > Destruction of RBCs in spleen, liver, RES due to alterations (reduced lifespan, 10-20 days) 4. Normal 5. Reticulocytosis/Polychromasia, Spherocytes 6. "Elevated EPO & Bilirubin, Abnormal osmotic fragility, Increased MCHC" 7. Asymptomatic: 20-30%. Symptomatic: Anemia, splenomegaly, jaundice/pigmented gallstones no hemoglobinemia/uria or hemosiderinuria 8. Splenectomy 9. Hemolytic Crises: Mononucleosis Aplastic Crisis: Parvovirus B19
Pyruvate Kinase Deficiency - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. AR, diminished glycolysis (ATP) > lose ATPase pumps, K+/H20 > deform and are removed 4. Normal 5. Reticulocytosis/Polychromasia, Echinocytes (burr cells) 6. Elevated EPO & Bilirubin, Increased 2,3 BPG 7. Anemia, significant hemolytic disease in newborns 8. Transfusion or Splenectomy 9. N/A
Alpha Thalassemias: 1 (silent carrier) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Decreased production of globin chains due to 1 deletion, more soluble tetramers with less severe hemolysis/more effective erythropoiesis than B-thal 4. Slight Microcytosis 5. Slight Microcytosis, Target Cells 6. Elevated EPO & Bilirubin, Electrophoresis abnormal only at birth with 2% Hg Bart (y4) 7. Asymptomatic 8. N/A 9. N/A
Alpha Thalassemias: 2 (trait) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Decreased production of globin chains due to 2 deletions (cis/trans), more soluble tetramers with less severe hemolysis/more effective erythropoiesis than B-thal 4. Slight Microcytosis 5. Slight Microcytosis, Target Cells 6. Elevated EPO & Bilirubin, Increased RBC, - Electrophoresis abnormal only at birth with 15% Hg Bart (y4), moderately decreased-normal Hg 7. Asymptomatic; minimal-no anemia 8. N/A 9. N/A
Alpha Thalassemias: 3 (Hg H disease) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Decreased production of globin chains due to 3 deletions, more soluble tetramers with less severe hemolysis/more effective erythropoiesis than B-thal. Extremely high O2 affinity 4. Microcytic, hypochromic 5. Target Cells 6. Elevated EPO & Bilirubin, Hg H (seen with Brilliant Cresyl Blue stain), decreased Hg 7. Moderate-severe anemia, most common in asia 8. Transfusion, bone marrow potential cure 9. N/A
Alpha Thalassemias: 4 (Hydrops fetalis) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Decreased production of globin chains due to 4 deletions, more soluble tetramers with less severe hemolysis/more effective erythropoiesis than B-thal 4. Microcytic, hypochromic 5. Target Cells 6. Elevated EPO & Bilirubin Hg Bart (y4), severely decreased Hg 7. Severe pallor, generalized edema, massive hepatosplenomegaly at birth. - Similar to hemorrhagic disease of the newborn. - Death in utero or shortly after without intrauterine transfusion. 8. Lifelong transfusion dependence, bone marrow potential cure 9. N/A
B-Thalassemia Intermedia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Decreased production of globin chains due to mutation in the beta chain (B+/B+ or Bo/B+) causes anemia due to decreased HgA and diminished survival due to a-chain precipitation 4. Microcytic, hypochromic 5. Target cells, nucleated RBCs, basophilic stippling 6. Elevated EPO & Bilirubin, Hg 6.0-9.5, MCV in 60s, increased RDW - Decreased HgA, Elevated HgA2 and F 7. Mild-Moderate Anemia 8. Transfusion and iron chelation, bone marrow transplant may be curative 9. N/A
B-Thalassemia Major - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Decreased production of globin chains due to mutation in the beta chain (Bo/Bo) causes anemia due to decreased HgA and diminished survival due to a-chain precipitation 4. Microcytic, hypochromic 5. Target cells, nucleated RBCs, basophilic stippling 6. Elevated EPO & Bilirubin, Hg 2.5-6.0, MCV in 60s, increased RDW - Little-no HgA, High HbF 7. Death in early childhood unless treated, massive erythroid hyperplasia > crewcut and chipmunk facies, hepatosplenomegaly, risk of aplastic crisis with ParvoB19, risk secondary hemochromatosis 8. Transfusion and iron chelation, bone marrow transplant may be curative 9. N/A
Hemoglobinopathy: Hemoglobin C Trait - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Inherit 1, Structurally abnormal globin chains synthesized > conformational changes in hemoglobin and RBC 4. Normal 5. Target cells, Normal reticulocyte count 6. Normal Hg levels - Electrophoresis: HbA 60%, HgC 40% 7. Asymptomatic 8. N/A 9. Increased resistance to Plasmodium Falciparum
Hemoglobinopathy: Sickle-Cell Trait - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Inherit 1, Structurally abnormal globin chains synthesized > conformational changes in hemoglobin and RBC 4. Normal 5. Target cells, RARE sickle cells, normal reticulocyte count 6. Sickle test+, Electrophoresis: HbA 60%, HgS 40%, May be elevated HgF/A2 7. Asymptomatic unless extremely hypoxic or dehydrated 8. N/A 9. Increased resistance to Plasmodium Falciparum
Hemoglobinopathy: Sickle-Cell Anemia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Inherit 2, Structurally abnormal globin chains synthesized > conformational changes in hemoglobin and RBC 4. Normal 5. Sickle Cells/Target Cells, Reticulocytosis/Polychromasia 6. HCT: 18-30%, Hyperbilirubinemia, elevated LDH1, Sickle test+ - Electrophoresis: HbS 85%+, No HbA 7. Severe Anemia with vaso-occlusive crises, autosplenectomy, and chronic organ damage (CHF, gallstones, bone infarct [fish mouth vertebrae], retinopathy, aseptic necrosis of femoral head, Salmonella Osteomyelitis. 8. Hydroxyurea 9. Potential aplastic or sequestration crises
Hemoglobinopathy: Hemoglobin SC Disease - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Inherit 2, Structurally abnormal globin chains synthesized > conformational changes in hemoglobin and RBC 4. Normal 5. Target cells, finger-like intracellular crystals, Reticulocytosis/Polychromasia 6. Elevated EPO & Bilirubin, Decreased Hg levels, sickle test+ - No HgA 7. Mild chronic anemia with splenomegaly (fewer complications than SS), retinopathy, ischemic bone necrosis are possible 8. N/A 9. N/A
Hemoglobinopathy: Hemoglobin C Disease - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Inherit 2, Structurally abnormal globin chains synthesized > conformational changes in hemoglobin and RBC 4. Normal 5. Target cells, hemoglobin C crystals, folded cells, Reticulocytosis/Polychromasia 6. Elevated EPO & Bilirubin, Decreased Hg levels - Electrophoresis: HbC 90%, HgF increased, No HgA 7. Mild chronic disease with fatigue, hemolysis and splenomegaly 8. N/A 9. N/A
Alloimmune AKA Transfusion reactions: Delayed - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Naturally occurring but not formed/very low titer alloantibodies (IgG) against foreign RBCs introduced via transfusion/pregnancy activate 4-6 days after. 4. Normocytic 5. Possible spherocytes 6. Elevated EPO & Bilirubin, Direct Coombs test+ 7. No Hemoglobinemia/hemoglobinuria, high bilirubin/urobilinogen 8. N/A 9. N/A
Alloimmune AKA Transfusion reactions: Hemolytic Disease of the Newborn (HDN) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. RBCs of fetus/newborn are destroyed by IgG antibodies that crossed the placenta (Erythroblastosis fetalis/Hydrops fetalis) 4. normocytic 5. Possible spherocytes 6. Elevated EPO & Bilirubin, Direct Coombs test+ 7. Anemia and Jaundice, Hepatosplenomegaly, Cardiac failure with marked edema and death. - Kernicterus 8. Tx - Prophylactic: Rhogam. - Antenatal: intrauterine transfusion, maternal plasmapheresis. - Postnatal: exchange transfusions, phototherapy. 9. Fetus can't conjugate bilirubin (no glucuronyl transferase). - ABO differences can be protective for developing.
Autoimmune AKA AIHA: Warm. Secondary - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Secondary autoantibodies (IgG) against self very common RBC antigens. Comorbid with: Lymphoproliferative disorders (CLL, thymoma, ect.), Collagen vascular disease, drugs, infectious disease, immunologic disease, GI disease, benign tumors. 4. Normal to Microcytic 5. Microspherocytes, Reticulocytosis/Polychromasia, schistocytes, nucleated RBCs 6. Elevated EPO, Bilirubin, LDH, and urine urobilinogen. Direct Coombs test+. - Rarely can have decreased haptoglobin with hemoglobinuria/hemoglobinemia. 7. Anemia jaundice/icterus,, gallstones unexplained fever, moderate splenomegaly. 8. Treat underlying disease. Avoid transfusions. Plasma exchange. Corticosteroids or Immunosuppressive. Splenectomy. 9. Patients serum agglutinates all reagent/donor cells. Optimal serum activity at 37C.
Glucose-6-Phosphate Dehydrogenase Deficiency - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular & Intravascular 3. X-LR, decreased activity in enzyme needed to prevent oxidative stress by reducing GSH - G6PD-: african, moderately reduced - G6PD mediterranean: middle eastern, significantly reduced 4. Normal 5. Reticulocytosis/Polychromasia, Heinz Bodies (oxidized/precipitated hemoglobin), bite cells 6. "Elevated EPO & Bilirubin, Abnormal osmotic fragility, Increased MCHC" 7. Self-limited Episodic hemolysis 2-3 days after oxidative stressor: drugs (antimalarials, sulfonamides, nitrofurans) Jaundice, Splenomegaly 8. N/A 9. N/A
Paroxysmal Nocturnal Hemoglobinuria (PNH) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Intravascular 3. Acquired defect in PIGA > deficiencies in CD55/59 which prevent spontaneous alternative complement pathway activation. Affects all hematopoietic cell lines 4. Normocytic 5. N/A 6. Pancytopenia, hemosiderinuria, hemoglobinuria, high bilirubin, low haptoglobin, negative direct coombs. Best confirmation by is by FC. 7. Mild-moderate anemia. 25% have hemoglobinuria in morning, chronic is more common triggered by many factors. - Thrombosis is leading COD. - Risk to develop other hematologic disorders: AML, MDS, aplastic anemia 8. Transfusion, iron supplementation and anticoagulation, steroids, bone marrow transplant 9. Consider when unexplained/unknown cause of: hemolytic anemia, iron deficiency anemia, hemoglobinuria, or pancytopenia with hypoplastic/aplastic bone marrow
Other traumatic hemolytic anemias - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Intravascular 3. Deposition of fibrin and platelets leads to mechanical injury of the RBCs as they pass through. - Can be due to cardiac valves, march hemoglobinuria, severe burns 4. Normal to Microcytic 5. Schistocytes, microspherocytes and polychromasia 6. Thrombocytopenia 7. N/A 8. N/A 9. N/A
B-Thalassemia Minor - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Decreased production of globin chains due to mutation in the beta chain (B+/B or Bo/B) causes anemia due to decreased HgA and diminished survival due to a-chain precipitation, 75% of cells die in marrow (ineffective erythropoiesis 4. Microcytic, hypochromic 5. Target cells, nucleated RBCs, basophilic stippling 6. Elevated EPO & Bilirubin, Hg 9.5-13.5, MCV in 60s, increased RBCs, increased RDW - Slightly decreased HgA, Elevated HgA2 (5%) and Hg F (2%) 7. Well adjusted, likely asymptomatic 8. Transfusion and iron chelation, bone marrow transplant may be curative 9. N/A
Autoimmune AKA AIHA: Warm. Primary/Idiopathic - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Autoantibodies (IgG) against self very common RBC antigens. Most common, insidious or sudden, may be precipitated by infection, trauma, surgery, pregnancy, stress. 4. Normal to Microcytic 5. Spherocytes, Reticulocytosis/Polychromasia, schistocytes, nucleated RBCs 6.Elevated EPO, Bilirubin, LDH, and urine urobilinogen. Direct Coombs test+. - Rarely can have decreased haptoglobin with hemoglobinuria/hemoglobinemia. 7. Anemia jaundice/icterus,, gallstones unexplained fever, moderate splenomegaly. 8. Remove initiating factors. Avoid transfusions. Plasma exchange. Corticosteroids or Immunosuppressive. Splenectomy. 9. Patients serum agglutinates all reagent/donor cells. Optimal serum activity at 37C.
Autoimmune AKA AIHA: Cold Agglutinin syndrome (acute) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Autoantibodies (IgM) against self very common RBC antigens bind in vascular beds with decreased temperature with sublytic but opsonizing quantities of complement. Usually due to infection (50% Mycoplasma), transient and self-limiting. 4. Normal to Microcytic 5. Reticulocytosis/Polychromasia. - RBCs agglutinate if slides not warmed. 6. Elevated EPO & Bilirubin, Direct Coombs test+. 7. By 2nd-3rd week (recovery of infection): rapid onset hemolysis, pallor, jaundice, splenomegaly. Raynaud's phenomenon. 8. Treat underlying disease. No therapy. Avoid cold weather 9. Patients serum agglutinates all reagent/donor cells. Optimal serum activity at 4C. Can be between 25-30.
Hemoglobinopathy: Hemoglobin E Trait - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Inherit 1, Structurally abnormal globin chains synthesized > conformational changes in hemoglobin and RBC 4. Normal 5. Target cells 6. Normal reticulocyte count Normal Hg levels - HgA 50%, HgE 50% 7. Asymptomatic 8. N/A 9. Increased resistance to Plasmodium Falciparum, risk of B-Thal/HgE phenotype
Hemoglobinopathy: Hemoglobin E Disease - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular 3. Inherit 2, Structurally abnormal globin chains synthesized > conformational changes in hemoglobin and RBC 4. Normal to Microcytic 5. Target cells and microcytes, Reticulocytosis/Polychromasia 6. Elevated EPO & Bilirubin, Decreased Hg levels - No HgA, HgE 95% 7. Mild anemia 8. N/A 9. Increased resistance to Plasmodium Falciparum, risk of B-Thal/HgE phenotype
Autoimmune AKA AIHA: Cold Agglutinin syndrome (chronic) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Extravascular and Intravascular 3. Autoantibodies (IgM, anti-I) against self very common RBC antigens bind in vascular beds with decreased temperature and activate complement. Usually idiopathic, maybe with lymphoma. 4. Normal to Microcytic 5. Reticulocytosis/Polychromasia - RBCs agglutinate if slides not warmed. 6. Elevated EPO & Bilirubin, Direct Coombs test+. Cold agglutination test+. Hemoglobinuria after significant cold exposure. - CBC difficult to obtain: may have falsely inflated (MCV, MCH, MCHC) and deflated (RBC, Hct) 7. Rarely severe and generally seasonal but can develop to a severe anemia. Raynaud's phenomenon 8. Treat underlying disease. No therapy. Avoid cold weather 9. Patients serum agglutinates all reagent/donor cells. Optimal serum activity at 4C. Can be between 25-30.
Autoimmune AKA AIHA: Paroxysmal Cold Hemoglobinuria (PCH) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Intravascular 3. Autoantibodies (IgG - autohemolysis (cold hemolysin or Donath-Landsteiner) anti-P) on RBC antigens mediates complement lysis. - Associated with syphilis and Children after a viral infection. Generally transient. 4. Normal to microcytic 5. Reticulocytosis/Polychromasia. Schistocytes, macrocytosis, microspherocytes 6. Elevated EPO, Bilirubin, and LDH. Direct Coombs test+. Decreased haptoglobin. 7. Acute, intermittent attacks of massive hemolysis after cold exposure. Hemoglobinuria after cold, fever/malaise, shaking/chills, back pain/cramps, abdominal pain, splenomegaly, renal insufficiency. 8. Steroid/transfusion as necessary. 9. Patients serum agglutinates all reagent/donor cells. Optimal serum activity at 4C. Can be between 25-30.
Microangiopathic hemolytic anemia (MAHA) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Intravascular 3. Deposition of fibrin and platelets leads to mechanical injury of the RBCs as they pass through. - Can be due to DIC, HUS (children with E. coli O157:H7 infection), and TTP. - Also: cancer, pre/eclampsia, malignant hypertension, SLE 4. Normal to Microcytic 5. Schistocytes, microspherocytes and polychromasia 6-7 HUS: thrombocytopenia and renal failure; TTP: thrombocytopenia, neurological signs, renal disease and fever 8. Cause 9 N/A
Alloimmune AKA Transfusion reactions: Immediate - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Hemolytic Anemias 2. Intravascular 3. Naturally occurring/preformed alloantibodies (IgM) against foreign RBCs introduced via transfusion/pregnancy activate. - Typically an ABO mismatch 4. Normocytic 5. N/A 6. Direct Coombs test+, Hemoglobinemia, hemoglobinuria 7. Shaking/chills, back pain, cramps, fever/malaise. Renal failure. Death 8. N/A 9. N/A
Pernicious Anemia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Autoimmune form of B12 deficiency prominent to develop at 60 in scandinavians 4. Macrocytic 5. Oval macrocytes, anisopoikilocytosis, fine basophilic stippling, Howell-Jolly bodies, decreased reticulocyte count, possible nucleated RBCs. Hypersegmented neutrophils, leuko/thrombocytopenia, karyorrhexis, large platelets 6. MCV > 110-120, Bone marrow findings: giant metamyelocyte (granulocyte), and large megakaryocytes, increased iron stores (ferritin) 7. Chronic atrophic gastritis due to autoimmune injury 8. N/A 9. N/A
Iron deficiency anemia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Chronic blood loss is the most common cause of iron deficiency in western world: ulcers, polyps, menses, repeated donations, diet (milk in infants, tea and toast in elderly). 4. Normal to Microcytic 5. Hypochromic, microcytic RBCs, anisopoikilocytosis with elliptocytes 6. Low MCV, high RDW, decreased serum iron, increased TIBC, decreased % sat, decreased ferritin, increased FEP. 7. Anemia, fatigue, lethargy, dizziness, pallor, alopecia, koilonychia, glossitis, pica, sideropenic dysphagia (difficulty swallowing), numbness/tingling in extremities 8. Treat underlying cause, oral if diet, IV/IM is severe or oral isn't tolerated. 9. Normal ferritin doesn't rule out. If inflammation is present the acute phase reactant lock-down of iron will result in a normal level.
Anemia of Chronic Renal Disease - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Chronic hemolytic/disease, bleeding and IDA damage kidney > decreased EPO secretion 4. Normal 5. Normochromic 6. N/A 7. Anemia proportional to the severity of the uremia. Uremia is the clinical picture of renal failure. 8. EPO effective, monitor iron levels to ensure max efficiency. 9. N/A
Lead toxicity - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Decreased erythropoiesis 4. Microcytic 5. Hypochromic: interfere with heme synthesis. Coarse basophilic stippling on peripheral smears. 6. Hemolysis: due to interference with membrane associated Na/K pumps. 7. Lead lines in bones, teeth, and other tissues. 8. Shows effects on brain/nerves, GI tract, bones, and kidney. 9. Think of when: unexplained anemia w/ basophilic stippling. Neurological changes in children, elevated lead, FEP, protoporphyrin, or delta-ALA.
Diffuse liver disease - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Diffuse liver disease, decreased folate, GI blood loss and IDA, lipid abnormalities 4. Macrocytic anemia 5. Target cells 6. N/A 7. N/A 8. N/A 9. N/A
Aplastic Anemia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Due to the:depletion of all hematopoietic stem cells by an agent or event which damages/kill stem cells, or immunosuppression of proliferation. - Most commonly are: idiopathic, due to chemical/drugs/irradiation, inherited disease, pregnancy, or PNH. 4. Normal - macrocytic 5. Pancytopenia 6. Dx requires bone marrow biopsy with markedly hypocellular to acellular marrow. 7. Insidious onset, no splenomegaly, symptoms due to bone marrow failure and pancytopenia 8. 60-70% respond to immunosuppression or bone marrow transplant. 9. Myelotoxic chemicals: arsenical, insecticides, benzene, chloramphenicol.
Sideroblastic anemia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Hereditary/congenital forms: ineffective erythropoiesis with increased bone marrow iron deposition, myelodysplasias (RARS) can be placed here. - Secondary causes: exposure to drug, alcohol, TB drugs 4. N/A 5. N/A 6. Bone marrow biopsy: ring sideroblasts, elevated serum iron concentration 7. N/A 8. 50% response to B6, removal of offending agent, iron chelation. 9. Isoniazid large cause along with chloramphenicol, phenacetin, and alkylating agents.
Hypothyroidism - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Hypothyroidism 4. Macrocytic anemia 5. N/A 6. N/A 7. N/A 8. N/A 9. N/A
Secondary hemochromatosis AKA hemosiderosis - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Iatrogenic iron overload, ineffective erythropoiesis syndromes, chronic liver disease, congenital atransferrinemia, neonatal hemochromatosis 4. N/A 5. N/A 6. N/A 7. N/A 8. N/A 9. N/A
Anemia of Chronic Disease - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Increased IL-6 increases hepcidin production (acute phase reactant), inhibits iron transfer from enterocytes to plasma and suppresses iron release from Mphages (lactoferrin) and inhibits EPO secretion. 4. Normal to microcytic 5. Normochromic-hypochromic 6. Decreased serum iron, TIBC, normal saturation, normal-increased ferritin 7. Mild anemia 8. Treat the underlying cause 9. N/A
Myelodysplastic Syndromes (Myelodysplasia) - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Ineffective erythropoiesis with increased bone marrow iron deposition 4. Macrocytic 5. N/A 6. Commonly; leukopenia and thrombocytopenia, cytogenetic changes in marrow precursors 7. N/A 8. N/A 9. Most commonly in elderly after chemo
Pure Red Cell Aplasia - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Inherited: Diamond-Blackfan anemia or idiopathic forms. -. Secondary forms: drug/virus induced (ParvoB19), thymoma, leukemia, autoimmune disease 4. Normal - macrocytic 5. N/A 6. Bone marrow biopsy is absent of RBC precursors, serum EPO increased 7. N/A 8. Thymic resection, immunosuppression, or plasmapheresis 9. N/A
B12 deficiency - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Megaloblastic anemias > impaired DNA synthesis leads to defective nuclear maturation and delayed division (dyssynchrony) leading to ineffective erythropoiesis and peripheral pancytopenia. - Due to pernicious anemia, diet, or poor absorption, fish tapeworm 4. Macrocytic 5. Oval macrocytes, anisopoikilocytosis, fine basophilic stippling, Howell-Jolly bodies, decreased reticulocyte count, possible nucleated RBCs. Hypersegmented neutrophils, euko/thrombocytopenia, karyorrhexis, large platelets 6. MCV > 110-120, Bone marrow findings: giant metamyelocyte (granulocyte), and large megakaryocytes, increased iron stores (ferritin). Increased MMA in urine. 7. Atrophic glossitis, subacute combined degeneration of the spinal cord, anemia, altered mental state, peripheral neuritis 8. B12 Oral/IM. - Not with folate, will hide the anemia. 9. Schilling test: abnormal is decreased excretion. If it increases when given intrinsic factor then that is the problem. If no change its malabsorption or an anti-IF antibody.
Folate deficiency - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Megaloblastic anemias > impaired DNA synthesis leads to defective nuclear maturation and delayed division (dyssynchrony) leading to ineffective erythropoiesis and peripheral pancytopenia. - Due to deficiency: poor diet (alcoholism, indigent, elderly, infancy), or increased loss (hemodialysis), or increased demand. 4. Macrocytic 5. Oval macrocytes, anisopoikilocytosis, fine basophilic stippling, Howell-Jolly bodies, decreased reticulocyte count, possible nucleated RBCs. Hypersegmented neutrophils, leuko/thrombocytopenia, karyorrhexis, large platelets 6. MCV > 110-121 7. No neurological symptoms 8. Folate 9. Methotrexate can cause, make sure its not a B12.
Myelophthisic anemias - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. Space occupying lesion in the marrow: cancer, granulomatous disease, leukemia/lymphoma, myelofibrosis > displace normal marrow 4. N/A 5. Teardrop cells (dacryocytes) 6. Leukoerythroblastosis (immature WBCs and nucleated RBCs) 7. N/A 8. N/A 9. N/A
Primary hemochromatosis - Type - Location - Cause - Cell Size - Blood Smear - CBC/Lab Findings - Symptoms - Treatment - Other
1. Impaired production 2. N/A 3. AR, HFE mutation (Cr6) action on hepcidin > increased iron absorption. 4. N/A 5. N/A 6. present in 50-60's: iron accumulates in parenchymal tissues > pancreas, liver, spleen, heart, skin. 7. Adult-onset diabetes "bronze-diabetes", cirrhosis, CHF 8. Phlebotomies and chelating agents 9. N/A
Lymphocytopenia (Lymphopenia) 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Leukopenia 2. No 3. Congenital immunodeficiency or acquired (HIV, steroids, cytotoxic drugs, autoimmune, malnutrition, some viral infections) 4. Lymphocytes 5. N/A 6. N/A 7. N/A
Agranulocytosis: Neutropenia 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Leukopenia 2. No 3. Less than 1000 neutrophils/uL. Most common cause: - drug toxicity (often a pancytopenia) predictably from chemotherapy/idiosyncratic (chloramphenicol & sulfonamide) - Possibly due: to ineffective granulopoiesis, inherited, immune-mediation, increased peripheral utilization, and hypersplenism. 4. Neutrophil 5. Bacterial/Fungal infections and ulcerations. Malaise, chills, fever, weakness, fatigue. - Bone marrow may be Hyper/hypocellular. 6. Hematopoietic growth factors, antibiotics, supportive care. 7. Can be overwhelming/fatal in hours/days. Worry if drops to <500/uL.
Specific signalling molecules for blood cells - G-CSF - M-CSF - IL-5 - IL-3/5
1. Neutrophil 2. Monocyte 3. Eosinophil 4. CD4+s'
Infectious Mononucleosis (lymphocytosis) 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Reactive Leukocytosis 2. No 3. Acute, self limited disease in teens due to B-cell lymphocytotropic EBV. 4. Atypical lymphocytes with dutch-skirting 5. Fever, fatigue, sore throat/pharyngitis, generalized lymphadenitis and splenomegaly, hepatitis, febrile rash. Decreased platelets/anemia. 6. N/A 7. No contact sports, may resemble a lymphocytic leukemia/lymphoma.
Basophilic Leukocytosis (basophilia) 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Reactive Leukocytosis 2. No 3. Common reaction to infectious/inflammatory stimuli, classified on basis of particular WBC affected. Could signal myeloproliferative disease (Chronic myeloid leukemia) 4. N/A 5. N/A 6. N/A 7. May mimic leukemia (leukemoid reaction). This occurs when WBC count is > 50,000.
Lymphocytosis 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Reactive Leukocytosis 2. No 3. Common reaction to infectious/inflammatory stimuli, classified on basis of particular WBC affected. Accompanies monocytosis associated with chronic immune response, viral infections, Bordetella pertussis. Neoplastic conditions. 4. N/A 5. N/A 6. N/A 7. May mimic leukemia (leukemoid reaction). This occurs when WBC count is > 50,000.
Neutrophilic Leukocytosis (neutrophilia) 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Reactive Leukocytosis 2. No 3. Common reaction to infectious/inflammatory stimuli, classified on basis of particular WBC affected. Acute (pyogenic) bacterial infections and tissue necrosis. 4. Toxic Change in PMNs: toxic granulation (larger and darker), Dohle bodies (puffy blue), cytoplasmic vacuoles. Reduced lobes (band-like) AKA left shift. 5. N/A 6. N/A 7. May mimic leukemia (leukemoid reaction). This occurs when WBC count is > 50,000.
Eosinophilic Leukocytosis (eosinophilia) 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Reactive Leukocytosis 2. No 3. Common reaction to infectious/inflammatory stimuli, classified on basis of particular WBC affected. Allergic disorders, parasitic infections (chagas), drug reactions. 4. N/A 5. N/A 6. N/A 7. May mimic leukemia (leukemoid reaction). This occurs when WBC count is > 50,000.
Monocytosis 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Reactive Leukocytosis 2. No 3. Common reaction to infectious/inflammatory stimuli, classified on basis of particular WBC affected. Chronic infections (TB, rickettsiosis, malaria) 4. N/A 5. N/A 6. N/A 7. May mimic leukemia (leukemoid reaction). This occurs when WBC count is > 50,000.
Reactive Lymphadenitis General 1. Type 2. Malignant? 3. Cause/description 4. Characteristic cell/pattern 5. Symptoms 6. Treatment/Prognosis 7. Other
1. Reactive Lymphadenitis 2. No 3. Immune mediated response to infectious agents 4. N/A 5. Tender lymphadenopathy 6. N/A 7. N/A
Platelets structure - peripheral zone - structural zone - organelle zone - membrane zone
1. cell membrane and glycocalyx with glycoprotein receptors 2. microtubules, actin filaments, myosin, and actin-binding proteins 3. mitochondria, peroxisomes, glycogen particles, and 3 types of granues - a granules: moderately dense - delta graunules: very dense - gamma granules: lysosomes 4. 2 types of channels: open canalicular system (OCS: for fast signaling/secretion), and dense tubular system (DTS: sequesters Ca for signaling)
delta-aminolevulinic dehydratase
2 d-aminolevulinic acid's > porphobilinogen - 1/4 heme - inhibited by lead
Hereditary coproporphyria
AIP w/ PCT rash - deficiency in coproporphyrinogen oxidase - coproporphyrinogen III and everything before builds up in urine - photosensitive -Painful abdomen - Port wine-colored urine - Polyneuropathy - Psychological disturbances - Precipitated by drugs, alcohol, and starvation - neurological issues
Porphyria cutanea tarda (PCT)
Caused by a deficiency in uroporphyrinogen decarboxylase - uroporphyrin accumulates in the urine - most common - patients photosensitive
Acute intermittent porphyria (AIP)
Deficiency in hydroxymethylbilane synthase AKA porphobilinogen deaminase - porphobilinogen and ALA accumulate - urine darkens when exposed to air - not photosensitive 5 P's: - Painful abdomen - Port wine-colored urine - Polyneuropathy - Psychological disturbances - Precipitated by drugs, alcohol, and starvation
Lead poisoning
Ferrochelatase and ALA dehydratase inhibited - protoporphyrin and ALA accumulate
Porphyrias
Hepatic (acute/chronic) or erythropoietic - AD - acute attacks precipitated by fasting or stimulation of heme synthesis - treat acute with hemin (Fe3+) and glucose
Myelocyte
Last mitotic stage - first stage of specific granules synthesis Tan coloring at golgi zone appears: neutrophil granules being made
Hemopoiesis
Mesoblastic: - yolk sac: 3 weeks - Hepatic: 6 weeks - splenic: 12 weeks Myeloid: - Marrow: 24 weeks Erythrocytes still have nuclei Leukocytes not present until 8 weeks
Granulopoiesis
Mitotic cell: - Myelocyte Post-mitotic cells: - Metamyelocyte - Band cell - Mature Neutrophil Nuclear changes - Large, euchromatic, transcriptionally active > condensed and inactive nucleus Cytoplasmic changes: - basophilic and abundant RER > pale-blue/pink cytoplasm, mature graunles
Erythroblast differentiation
Mitotic: - Proerythroblast - Early erythroblast: basophilic erythroblast - Middle erythroblast: polychromatophilic erythroblast Post-mitotic: - Late erythroblast: orthochromatophilic erythroblast/normoblast - Reticulocyte and Erythrocyte Nuclear changes - Large, euchromatic, transcriptionally active > absent Cytoplasmic changes: - basophilic and abundant RER > acidophilic with abundant hemoglobin
Metamyelocyte
Nucleus changing shape, no longer spherical specific granules present
Neutrophils
Specific granules: less dense Non-specific granules: more dense (azurophilic) Multi-lobed nucleus
Promyeloblast
azurophilic granules formed golgi ghost largest granulocytic precursor in marrow
Erythroid islet
central Mphage surrounded by erythroblasts will eat the kicked out nucleus and recycle aged RBCs
Hb chesapeake
decreased ability to deliver o2 to tissues (increased o2 affinity) due to mutated aB contacts. - induced polycythemia due to EPO induction
hereditary orotic aciduria
defect in UMP synthase - orotic acid builds up - growth retardation, severe megaloblastic anemia Tx: uridine helps decrease build up ornithine transcarbamylase def. can lead to orotic acid build up because the increased carbamoyl phosphate bypasses regulation from UMP synthase leading to increased orotate. - no anemia - ammonemia
post-hepatic jaundice
extrahepatic biliary obstruction (cholestasis) excess conj. bilirubin stool pale Alkaline phosphatase elevated dark urine
Myeloblast
first recognizable precursor in granulocytic series - high nuclear-to-cytoplasmic ratio
polychromatophilic erythroblast
gray cytoplasm last mitotic stage
Eosinophil
had Charcot-leyden protein: bipyramidal crystals associated with eosinophilic inflammatory reactions and asthma Bi-lobed nucleus specific Granules: dark crystalloid body of major basic protein (MBP)
pre-hepatic jaundice
hemolytic issues increased unconj. bilirubin
Band/stab cell
immature granulocyte with horseshoe shaped nucleus
physiological jaundice of the newborn
immaturity of bilirubin conjugating system > increased unconjugated bilirubin in serum > irreversible brain damage (kernicterus) Tx: blue light or phenobarbitol
Lymphocytes
large amount of dense heterochromatin finger-like microvilli
Proerythroblast
large basophilic golgi ghost
Basophil vs mast cell
mast cell larger, mononuclear with smaller and more numerous cytoplasmic granules
hepatic jaundice
mixed conj. and unconj. bilirubin often due to damage of the liver (viral hepititis) Liver enzymes elevated (ALT, AST) dark urine
reticulocyte
no DNA residual rRNA material present when viewed after a supravital stain
Methemoglobinemia (HbM)
o2 binding pocket mutated, Fe oxidized to Fe3+ and doesn't bind o2 - chocolate cyanosis
serum feroxidase (ceruloplasmin)
oxidized Fe2+ > 3+ so that it can leave the mucosa and be brought into cells
Basophilic erythroblast
peroxidase staining reveals hemoglobin being synthesized
biliverdin reductase
produces bilirubin (yellow)
heme oxygenase
produces biliverdin (green) - release iron - molecular O2 used and results in CO formation
ferrochelatase
protoporphyrin IX + iron > heme - inhibited by lead
adventitial cell/reticular cells
provides support for the developing blood cells and produce reticular fibers stimulate differentiation by secreting several cytokines
orthochromatophilic erythroblast/normoblast
reddish-blue cytoplasm - nucleus condensed, featureless and eccentrically positioned
delta-aminolevulinic acid (ALA) synthase
succinyl CoA + glycine > d-aminolevulinic acid (ALA) synthase - need B6 Rate limiting - erythroid type: active in the presence of iron - housekeeping type: in most tissue (liver) inhibited by heme, activated by EPO.
