Hematology Practice Exam 2

Which of the following laboratory results characterize(s) iron-deficiency anemia? Please choose ALL that apply.

Decreased MCHC, MCH, Hemoglobin, MCV.

Which of the following is not characteristic of hereditary xerocytosis?

Decreased MCV and decreased MCHC

Which of the following is not characteristic of hereditary hydrocytosis (stomatocytosis)?

Decreased MCV and increased MCHC Is a characterist: Increase MCV, decreased MCHC Inherited autosomal dominant Increased passive permability of sodium Stomatocytes

Which of the following is characteristic of anemia of inflammation?

Decreased red blood cell life span Impaired iron metabolism Suppression of erythropoiesis Decreased erythropoiesis levels

Which of the following is characteristic of the vaso-occlusive or painful crisis in a patient with sickle cell anemia? Please choose ALL that apply.

Dehydration or exposure to extreme cold Severe pain Tissue damage and necrosis Regional hypoxia and acidosis

Which of the following is characteristic of HE?

Demonstrates a biochemical and genetic relationship to HPP; Demonstrates variable linkage to the Rh gene; Elliptocytes in the peripheral blood (>30%); Most often inherited autosomal dominant

A screening laboratory test for Paroxysmal Cold Hemoglobinuria is:

Donath-Landsteiner Test

Paroxysmal Cold Hemoglobinuria

Donath-Lansteiner test

What recombinant cytokine has proven effective in counteracting the suppressing effects of IL-1 in AOI (anemia of inflammation)?

EPO (Erythropoietin)

Hereditary Hemochromatosis is due to an abnormal HFE gene on Chromosome #6, which regulates Hepcidin production. Which of the following would you expect to find in this anomaly? Please choose ALL that apply.

Enterocyte iron absorption increased Leading to fibrosis Hemosiderosis of RES Bronze diabetes

Which of the following represents hereditary intracorpuscular defects?

Enzyme deficiencies Thalassemia RBC membrane defects Hemoglobinopathies

Favism, neonatal jaundice, and congenital nonspherocytic hemolytic anemia are all exacerbating clinical conditions associated with G6PD deficiency. What type of hemolysis can we expect with these?

Extravascular

Which of the following is the major type of hemolysis characteristic of the majority of cases of HS?

Extravascular hemolysis

Which of the following is the intracellular storage form of iron?

Ferritin

Paroxysmal nocturnal hemoglobinuria is a rare hemolytic anemia caused by a mutated PIGA gene, which codes for the GPI anchor which anchors proteins to the cell surface. Without this GPI anchor, cell surface proteins are missing from the cell. What else if true of PNH (paroxysmal nocturnal hemoglobinuria)? Please choose ALL that apply.

GPI deficiency leads to increased complement-mediated hemolysis; RBC lysis is exacerbated by infection, surgery, and transfusion, Patient notices Hemoglobinuria in the morning; This disease also affects WBCs and platelets; Intravascular lysis is primary manifestation; Patient can demonstrate anemia, leukopenia, and thrombocytopenia.; Anemia is mostly normocytic/normochromic. But slight macrocytosis and polychromasia may be present.

The alpha thalassemias are caused by __________, while the beta thalassemias are caused by _____________________.

Gene deletion; gene mutation

Heinz bodies may be seen in what hematologic disease/s?

Glucose 6-phosphate dehydrogenase (G6PD) deficiency Alpha thalassemia Unstable hemoglobinopathies

Paroxysmal nocturnal hemoglobinuria

Ham's test

The performance of this screening test for Paroxysmal Nocturnal Hemoglobinuria (PNH) involves placing red cells in mild acid to test the RBC fragility. What test is this?

Ham's test

Which of the following hemoglobin(s) is/are present in hemoglobin SC disease? Please choose ALL that apply.

HbC HbS

Which red cell inclusion is characteristic of G6PD deficiency?

Heinz bodies

What disease state can be assessed by hemoglobin electrophoresis?

Hemoglobinopathies

Which of the following tests reflect an increase in red cell destruction?

Hemoglobinuria Hemosiderinuria Bilirubinemia Hemoglobinemia

Staining the urine sediment for iron with Prussian blue will detect which of the following during hemolytic anemia?

Hemosiderin

A 40-year-old patient was admitted to the hospital with acute cholecystitis and a palpable spleen. A CBC was performed: Hemoglobin, 13.0 g/dL; MCV, 80 fL; Hct, 38%; MCH, 28 pg; Retic, 7%; MCHC, 36%; RPI, 3.9; Coombs' test negative; spherocytes, 2+; anisocytosis, 2+; poikilocytosis, 2+. Which of the following disorders is consistent with the laboratory profile?

Hereditary spherocytosis (HS)

Considering the following: Hematocrit 28%. Chronic anemia. Moderate splenomegaly, Retinal hemorrhages. Chest pain. Peripheral smear that reveals quarter-moon shaped RBCs with pointed projections. Which of the below choices is the most likely diagnosis?

Hgb S-S

Which of the following red cell inclusions may be seen in the RBCs of patients with sickle cell anemia as a result of rapid RBC turnover and "stressed" erythropoiesis? Please choose ALL that apply.

Howell-Jolly bodies Siderotic granules

Chronic blood loss in a patient with malignancy results in __________ anemia.

Hypochromic

A patient with microcytic anemia might have which of the following diseases?

IDA Thalassemia Sideroblastic anemia Lead poisoning

Which cytokines are thought of as key pathogenetic factors in AOI (anemia of inflammation)?

IL-1

Which of the following laboratory results are characteristic of HS?

Increased MCHC Abnormal osmotic Abnormal autohemolysis Normal MCV

Which of the following characterizes hereditary spherocytosis (HS)?

Increased MCHC Decreased surface-to-volume ratio Spherocytes Hemolytic anemia

In the anemia of chronic disease/inflammation, there is an increase in the erythrocyte sedimentation rate (ESR) due to:

Increased fibrinogen

GI bleed, menorrhagia, proton pump inhibitor medications are all most closely associated with which of the following?

Iron deficiency anemia

In the anemia of inflammation it is suggested that one of the reasons for a decreased transferrin saturation may be:

Iron is blocked in the fixed macrophages of the RES

One-third of the total body iron may be found in storage pools such as:

Liver Bone marrow Spleen

Which of the following laboratory findings may be characteristic of sickle cell anemia? Please choose ALL that apply.

Marked erythroid hyperplasia Thrombocytosis Neutrophilic leukocytosis with a shift to the left An average reticulocyte count between 5% and 20%

Popeye has a deficiency of vitamin B12 and folic acid. His RBCs have an oval macrocytic appearance. Which of the following does Popeye most likely suffer from?

Megaloblastic anemia

Hereditary Spherocytosis is best described as which of the following?

RBCs with spectrin and ankrin deficiency. These cells experience a greater than normal influx of Na+, and are at risk of osmotic swelling which causes them to burst due to their decreased surface:volume ratio.

Which of the following is characteristic of PK deficiency?

Clinical manifestations commonly found include splenomegaly and cholelithiasis; The severity of the hemolytic anemia varies widely; Varying degrees of polychromasia and poikilocytosis may be found on the peripheral smear and nucleated red cells may be seen; Onset may be during infancy or early childhood and mild cases may not be detected until adulthood

With more hematologically affected thalassemia patients, the BM is trying to compensate for lack of adequate globin chain synthesis in hemoglobin. As a result, the BM expands to extramedullary regions. What typical physical changes can this cause?

-

In a normal individual, how many genes encode for the alpha globin chain?

4.

Approximately what percent of ingested iron is absorbed?

5-10%

In the Donath Landsteiner Test:

the anti-P antibody attaches to the P antigen at colder temperatures, and the RBC lyses when the cells reach warmer temperatures.

Transferrin is:

A protein in blood plasma that carries iron absorbed through duodenum and jejunum to the liver, spleen, and bone marrow.

Abnormal lipid metabolism causes which of the following: Please choose all that are correct.

Acanthocytes Macrocytes Target cells

This condition allows the individual to be hematologically normal, or slightly microcytic. It is an underlying single α chain gene deletion. What is it?

Alpha Thalassemia Silent Carrier

A Delta check of patient hemoglobin values helps with:

Anemia due to improperly collect lab samples

Transfer of storage iron to erythroid precursors within the BM is blocked by the fixed macrophages of the RES in this hypoproliferative anemia. This causes shortened RBC survival and failure of the BM to increase RBC production. It can be caused by infection, inflammation, malignancy, chronic rejection after solid organ transplantation, and acute illness.

Anemia of chronic disease

In which of the following anomalies would you expect the patient to have pancytopenia? Please select ALL that apply.

Aplastic anemia Paroxyxmal Nocturnal Hemoglobinuria Fanconi's Syndrome

Multiple infarctions and subsequent fibrosis of the spleen lead to what process in adult patients with sickle cell anemia?

Autosplenectomy

Which of the following most closely represents megaloblastic anemia?

B Notice the macro-ovalocytes and hypersegmented neutrophil. These are classic for Megaloblastic Anemia.

Which of the diseases below are characterized by the following? Little or no β chain synthesis No Hgb A synthesized Majority (up to 90%) of hemoglobin is Hgb F (α2, γ2)

Beta thal major

The anemia of chronic disease and inflammation can be differentiated from iron-deficiency anemia by which of the following?

Bone marrow iron stores

In Megaloblastic Anemia, the nuclei of the bone marrow RBC precursors are less condensed, and the chromatin is open-weaved in comparison to its more voluminous (older looking) cytoplasm. Condensation of nucleus is disrupted due to Vit B12, folate deficiency. This leads to nuclear fragmentation and destruction of fragile cells. This is called Asynchrony. Which of the images below represents Asynchrony?

C

Once a hemolytic anemia is determined, which of the following tests would rule out an immune hemolytic anemia if negative?

Coombs' test (DAT)

Which of the following would you expect in Beta Thalassemia Minor?

this disease mimics IDA. Microcytic/hypochromic smear. Except in Beta thal, the RBC count can be increased in an attempt to compensate for the funky hemoglobin.

Which of the following is characteristic of HPP? Please choose all that apply.

Microspherocytosis Thermal instability of red cells when heated to 45 C in vitro Partial deficiency of spectrin RBC fragmentation & micropoikilocytosis

Which of the following is characteristic of HbC disease? Please choose ALL that apply.

Mild chronic hemolytic anemia Normocytic, normochromic anemia with numerous target cells (50-90%) and occasional folded cells Splenomegaly Hemoglobin C ("bar of gold") crystals present on peripheral smear of splenectomized patients

Which of the following laboratory results is not characteristic of HPP?

Normal MCV. Is a result: low mcv, increase in osmotic fragility, RBC thermal instability, increase in autohemolysis test

Which of the following laboratory results is characteristic of the common type of HE? Please choose ALL that apply.

Normal autohemolysis test Greater than 75% elliptocytes Normal RBC indices Normal osmotic fragility

Which of the following lists of laboratory findings would be most characteristic of the anemia of chronic disease?

Normocytic, normochromic anemia; low serum iron level; decreased TIBC; increased ferritin levels

This alpha and beta spectrin deficiency (mutation) causes impairment of spectrin dimer association. This disease demonstrates red cell budding, red cell crenation, rare elliptocytes, and spherocytes in circulation. These RBCs can begin to fragment at 37oC (body temp), while normal RBCs fragment at 49oC. CBC parameters demonstrate: Hemoglobin: <6.5 g/dl, Low MCV 50-75 fL, MCHC Usually elevated

Notice the elliptocytes, small spherocytes, and schistocytes. These cells are at increased susceptibility of spectrin degradation.

Hereditary Spherocytosis

Osmotic fragility test

Which of the following hematological results will suggest an individual with sickle cell trait? Please select ALL that apply.

Peripheral blood smear is normal with the exception of target cells Hemoglobin electrophoresis demonstrates hemoglobin A, hemoglobin S, and usually slightly elevated hemoglobin A2 Solubility screening tests are positive

Which of the following is caused by either anti-parietal cell antibodies or anti-intrinsic factor antibodies?

Pernicious anemia

This type of anemia is not an anemia. It is caused by incorrectly collected or prepared blood sample. "Anemia" is normocytic/normochromic, which also demonstrates decreased WBCs and platelets. Delta checks can prevent this type of "anemia."

Preanalytical anemia

Deficiency of this enzyme in the Embden-Meyerhof pathway causes a right shift in the oxygen dissociation curve due to increased 2.3-DPG production.

Pyruvate kinase

When RBCs leak Na+ and K+ ions through unregulated ion channels, and this leads to a net loss of K+:

RBCs become dehydrated and Hgb puddles at one end of the cell, forming a xerocyte

Pernicious Anemia

Schilling's test

Which of the following remains the major cause of morbidity and mortality in patients with sickle cell anemia?

Serious bacterial infections

Which of the following classic hematologic features of sickle cell anemia can be observed on the peripheral blood smear?

Sickle cells Nucleated red cells Target cells Anisocytosis and poikilocytosis

Which of the following disorders is caused by iron being trapped in the mitochondrion of the cell resulting in a problem in forming the RBC hemoglobin molecule?

Sideroblastic anemia

The morphologic hallmark of HS hereditary spherocytosis on the peripheral blood smear is:

Spherocytes

Which of the following is the most common treatment for most patients with HS?

Splenectomy

Which of the following represents quantitative hemoglobinopathies characterized by a decreased production of hemoglobin with a decreased synthesis of one particular globin chain?

Thalassemia

Aplastic anemia manifests with pancytopenia. It is caused by a stem cell auto-antibody, chemical agents, drugs, infections. It causes decreased BM cellularity and reticulocyotisis. Which of the following morphologies would you expect for Aplastic Anemia?

normocytic/normochromic RBCs, decreased WBCs and platelets

The diagnosis of G6PD deficiency in a black man during acute hemolysis may be difficult to detect in a screening test due to:

The increased number of younger red cells (reticulocytes) that have normal levels of G6PD and a lack of older G6PD-deficient red cells due to hemolysis

Which of the following is/are indicative of iron deficiency anemia? Please choose ALL the apply.

pica, cheilitis, and koilonychias RBC 2.0 Hgb 6.0 g/dL, Hct 18%, MCV 68 fL with increased RDW microcytic/hypochromic anemia BM iron depletion

Which of the following amino acid substitution occurs in hemoglobin S?

Valine for glutamic acid

The below picture shows asynchronous development of RBC precursors in the BM in the top image. The bottom images shows a normal normoblast. What is the probable cause of the asynchronous development of the RBC precursor in the top image?

Vit B12 and folate deficiency

Sideroblastic anemia is either inherited (5-aminolevulinic synthetase enzyme defect) or acquired (lead poisoning, alcohol, drugs, chronic transfusions). Which of the following would you NOT expect to find in association with this disease?

normochromic macrocytes on peripheral smear