Heme Exam

If the serum iron was abnormally low and the TIDC slightly elevated, what would you conclude about the state of iron storage in the bone marrow?

Iron stores are absent

The elliptocyte is a prominent morphology in:

Iron-deficiency anemia

What is the definition of pure red cell aplasia?

Lack of erythroid precursors with normal white blood cell and megakaryocyte precursors

.What is (are) the main components of the flow cytometer?

Laser light source b. Fluidics c. Photodetectors

What is the correct list for the number and type of globin chains in normal adult hemoglobin?

2 alpha, 2 nonalpha chains

What is the composition of normal adult hemoglobin?

95-97% Hgb A, 2-3% Hgb A2; 1-2% Hgb F

How is aplastic anemia best defined?

A condition in which bone marrow production of red blood cells, white blood cells, and platelets has failed

A microcytic cell can be described as possessing:

A size of less than 7 u

The WBC is 15 x 10(9)/L with 90% blasts, Q% segs, and 4% monos. The blasts are relatively large and have abundant cytoplasm. Over 90% of them are positive with the nonspecific esterase stain and an occasional blast is positive with the Sudan black. What is the diagnosis?

AML, M5 type

The WBC is 50 x 10(9)/L with 80% blasts, 15% segs, and 5% lymphs. The bone marrow reveals sheets of immature cells. Cytochemical studies of these cells show that they are peroxidase-positive (20%) and the nonspecific esterase is negative. What is the diagnosis?

AML, Ml type

Each of the following favors a diagnosis of chronic myelogenous leukemia (CML) rather than a leukemoid reaction EXCEPT:

Absence of eosinophils and basophils in the peripheral blood THESE ARE Low LAP score with myeloblasts through segs in the peripheral blood c. Philadelphia chromosome d. Enlarged spleen

Which statement best describes paroxymal nocturnal hemoglobinuria (PNH)?

Acquired hemolytic anemia associated with cellular membrane abnormalities

Which of the following is not a characteristic of hemoglobinopathies?

All are manifested in clinically significant conditions THESE ARE Conditions in which abnormal hemoglobins are synthesized b. Result from inherited abnormalities or genetic mutations Result in a defect in structural integrity of function of the hemoglobin molecule

What is the process in which the immune system produces antibodies to foreign red cell antigens introduced into their circulation through transfusion, pregnancy, or organ transplantation?

Alloimmune hemolytic anemia

Which of the following influence(s) iron absorption?

Amount and type of iron in food b. Function of GI mucosa and pancreas c. Erythropioetic needs and iron stores

What is meant by "gating"?

An electronic window separating one subpopulation of cells

What is true concerning delayed hemolytic transfusion reactions?

Antibodies are usually IgG formed from a secondary response

What is the principle of the Donath-Landsteiner test?

Antibody binds red cells at 4C and causes lysis at 31C

What immune system abnormality results in the loss of self-recognition for an individual's own red cell antigens?

Autoimmune hemolytic anemia

Which granulocytic cell has large, abundant purple-black granules?

Basophil

What is a characteristic finding in lead poisoning?

Basophilic stippling

What is the enzyme deficiency seen in Gaucher's disease?

Beta-glucocerebrosidase

How is hematocrit determined on automated hematology instruments?

Calculation (MCV X RBC count)

What is the most commonly accepted method of measuring hemoglobin?

Conversion of hemoglobin to cyanmethemoglobin followed by spectrophotometric measurement

What are the characteristics of Niemann-Pick cells?

Cytoplasm filled with lipid droplets, inconspicuous nucleus

Directional migration toward a gradient stimulated by a chemoattractant is referred to as:

Chemotaxis

Malignant lymphomas may best be differentiated from infectious mononucleosis by which of the following features?

Clonality b. Monotony c. Pattern of lymphadenopathy

At what stage of differentiation will cells become committed to one cell line?

Colony-forming unit (CFU)

What factors contribute to the sickling of RBCs?

Decrease in pH and oxygenation and dehydration

Which of the following laboratory tests would not be typical of hereditary spherocytosis?

Decreased MCHC THESE ARE Increased osmotic fragility b. Spherocytes on peripheral smear Increased reticulocyte production index (RPI)

Which of the following laboratory results would not be a usual diagnostic criterion for a patient with anemia?

Decreased platelet level THESE ARE Decreased hemoglobin level b. Decreased hematocrit level Decreased RBC level

Which of the following would not be seen in sideroblastic conditions?

Decreased serum iron THESE ARE Increased RDW Pappenheimer bodies Ringed sideroblasts

The pathophysiology of megaloblastic anemia is:

Defective DNA synthesis and abnormal nuclear maturation

What is the hemoglobin defect found in thalassemia syndromes?

Defective production of the globin portion

The defect in chronic granulomatous disease is attributed to:

Deficiency or defect in cytochrome b

What is the diagnostic value of the reticulocyte count in the evaluation of anemias?

Determines response and potential of the bone marrow

Which metabolic pathway generates 90% of the ATP needed by RBCs?

Embden-Meyerhof pathway

Infectious mononucleosis is caused by which of the following?

Epstein-Barr virus

What infectious agent is most commonly associated with the pathogenesis of Hodgkin's disease?

Epstein-Barr virus

What condition is defined by a platelet count >600 x 10(9)/L, megakaryocytic hyperplasia, absence of Philadelphia chromosome: and hemoglobin ≥ 13 g/dl (or normal red cell mass)?

Essential thrombocytosis

How is laboratory diagnosis made for malarial infection?

Examination of peripheral blood smear

Which response represents the most complete and correct listing of the most common clinical signs of anemia?

Fatigue, weakness, gastrointestinal symptoms, dyspnea, pallor

The morphological characteristic(s) associated with Chediak-Higashi syndrome is/are:

Giant lysosomal granules

Oxidative denaturation of hemoglobin results in formation of small particles that are visualized with supra-vital staining. What is the term for these particles?

Heinz bodies

Which inclusion cannot be visualized on Wright's stain?

Heinz bodies

What term refers to the accumulation of excess iron in macrophages?

Hemosiderosis

Which of the following is not a crucial area of RBC survival and function?

Intravascular hemolysis THESE ARE Integrity of RBC cellular membrane b. Cell metabolism Hemoglobin structure

The glycoprotein necessary for absorption of vitamin Bl2 is:

Intrinsic factor

Which list represents the complete set of processes necessary for normal hemoglobin production?

Iron delivery and supply, synthesis of protoporphyrins, globin synthesis

Reactive lymphocytes may best be distinguished from blasts by the presence of which of the following morphological features?

Heterogeneous cell population

What is the enzyme deficiency in Tay-Sachs disease?

Hexosaminidase A

What are the characteristics of Gaucher's cells?

Histiocytes with blue, folded cytoplasm

Which type of thalassemia has primarily hemoglobin Bart's and shows the following clinical expressions: infants die in utero or soon after birth; severe anemia; marked hepatomegaly; and splenomegaly; and ascites?

Homozygous alpha thalassemia

Which type of red blood cell inclusion is a DNA remnant?

Howell-Jolly bodies

What is the primary risk to thalassemia major patients who are on a high-transfusion (hypertransfusion) program?

Iron overload

What is the appearance of the bone marrow biopsy in aplastic anemia?

Hypocellular

Pelger-Huet anomaly may be characterized by:

Hyposegmentation of nucleus with most neutrophils bilobed or monolobed

Which of the following is not seen on the peripheral smear of a patient with megaloblastic anemia?

Hyposegmented neutrophils THESE ARE Macro-ovalocytes Hypersegmented neutrophils d. Howell-Jolly bodies

Which is the most common cause of aplastic anemia?

Idiopathic

Which of the following is a proposed mechanism for drug-induced hemolytic anemia?

Immune complex b. Drug absorption (hapten) c. Membrane modification

Which of the following is not characteristic for immediate hemolytic transfusion reactions?

Increase in plasma haptoglobin THESE ARE Acute intravascular hemolysis b. Most commonly caused by ABO IgM complement-activating antibodies Hemoglobinemia, hemoglobinuria, and hemosiderinuria

What are the laboratory findings in polycythemia vera?

Increased hemoglobin and/or hematocrit, increased RBCs, granulocytes, and platelets

What are causes for nonimmune hemolytic anemia?

Infections b. Mechanical, chemical, and physical agents c. Acquired membrane disorders

What is the correct sequence for iron transport?

Ingestion, conversion to ferrous iron in stomach, reconversion to ferric state in bloodstream, transport by transferrin, incorporation into cells and tissues

Organs primary hematopoiesis in fetus

Liver, spleen, BM

Which features are the most important characteristics of chronic myelogenous leukemia that distinguish it from myelofibrosis?

Low LAP score and presence of Philadelphia chromosome

What are the two major categories of iron deficiency?

Low availability and increased loss of iron

Which cell classification is described by the following: second most abundant cell in the blood of a normal adult, usually small and round; sky-blue to intensely blue cytoplasm; and nucleus with clumped dark purple chromatin?

Lymphocyte

Which cell may be found in mucopolysaccharide disorders?

Lymphocytes with Alder-Reilly bodies

How would a red blood cell that has a diameter of 9 u and an MCV of 104 In (3) be classified?

Macrocyte

The type of anemia most commonly encountered in myelodysplastic syndrome (MDS) is:

Macrocytic, normochromic

According to the morphological classification of anemias, megaloblastic anemia is a:

Macrocytic, normochromic anemia

What causes hemolytic disease of the newborn (HDN)?

Maternal IgG antibodies cross the placenta, formed as a result of a previous blood exposure and/or pregnancy, cross the placenta and attach to fetal cells

Which granulocyte has a kidney-bean shaped nucleus with a clumped chromatin pattern and small secondary granules with a few primary granules?

Metamyelocyte

What is the anemia classification of thalassemia?

Microcytic, hypochromic anemia

What condition is not a cause of anemia?

Moderate exercise THESE ARE Decreased RBC production d. Increased RBC destruction or los Dietary deficiency

What is the proper cell sequence for the monocytic-phagocytic system (MPS)?

Monoblast, promonocyte, monocyte, macrophage

What is the "gold standard" for classifying lymphomas and leukemias?

Morphological classification

Which of the following organisms ate associated with hemolytic anemia?

Mycoplasma pneumonia b. Epstein-Barr virus c. Babesia microti

Cell sequence of granulopoeisis

Myeloblast, promyelocyte, myelocyte, metamyelocyre, band, neut

What deficiency causes hemoglobin to be oxidized from the ferrous to the ferric state?

NADH-methemoglobin reductase deficiency

What is the origin of the myeloproliferative disorders?

Neoplastic transformation of multipotential stem cells

What nonspecific laboratory findings are characteristic for PNH?

Normocytic, normochromic anemia; leukopenia with decreased granulocytes; low LAP; thrombocytopenia; urine hemosiderin

What is the predominant abnormal characteristic erythrocyte morphology associated with idiopathic myelofibrosis?

Ovalocytes

What is the primary function of iron?

Oxygen transport

Which of the following is a correct description of the sugar water test (sucrose hemolysis test)?

PNH cells are lysed by complement-after exposure to low-ionic-strength sugar water

Which is not a characteristic of warm autoimmune hemolytic anemia?

Positive result for Donath-Landsteiner test THESE ARE Variable anemia b. Reticulocytosis and spherocytosis DAT result usually positive for both IgG and C3d

What is the treatment for malaria?

Quinine sulfate

What steps occur in the intravascular breakdown of senescent RBCs?

RBCs breakdown in lumen of vessel; haptoglobin-hemoglobin complex goes to liver; unbound hemoglobin dimers are excreted through kidney as hemosiderin, hemoglobin or methemoglobin; haptoglobin is broken down to be excreted as urobilinogen

What steps occur in the extravascular breakdown of senescent RBCs?

RES cells phagocytize red cells; iron is coupled to transferrin and returned to bone marrow; globin is returned to amino acid pool; biliverdin is converted to bilirubin; bilirubin is coupled to albumin and transported to liver; bilirubin, glucoronide is converted to urobilinogen and excreted.

Basophilic stippling is composed of:

RNA

The diffuse aggressive lymphomas are:

Rapidly fatal if untreated

The cell characteristic of all types of Hodgkin's disease is the:

Reed-Sternberg cell

Which of the following cells is caused by iron accumulation?

Ringed sideroblast

Which of the following would not be characteristically found on a peripheral blood smear in the case of anemia?

Rouleaux and Dohle bodies THESE ARE Anisocytosis and/or poikilocytosis b. Basophilic stippling, Howell-Jolly bodies, and Pappenheimer bodies c. Cabots rings and Heinz bodies

Cell sequence of erythropoesis

Rubiblast, prorubicyte, rubicyte, metarubicyte, retic, erythro

The blood smear of a patient with a prosthetic heart valve may show:

Schistocytes

Which of the following represents the most complete list of causes for aplastic anemia?

Secondary, idiopathic and congenital

What are the next laboratory tests that need to be performed for IDA

Serum iron and TIBC

Which abnormal RBC is not caused by a structural membrane defect?

Siderocytes THESE ARE Spherocytes b. Target cells Acanthocytes

In a patient with an MCHC >36%, one would expect to observe:

Spherocytes

What is the enzyme deficiency seen in Niemann-Pick disease?

Sphingomyelinase

What defect is found in lipid storage diseases?

Subcellular accumulation of unmetabolized material in lysosomes

Which morphologies would be prominent on a smear of a patient with severe liver disease?

Target cells, macrocytes

The staging system for Hodgkin's and non-Hodgkin's lymphomas is:

The Ann Arbor staging system

How is a monoclonal antibody produced?

The fusion of immune B cells with a myeloma partner to produce an immortalized hybridoma cell producing a unique antibody

What is the definition of flow cytometry?

The measurement of cellular (or particle) properties as they move in a fluid (flow) past a stationary set of detectors

What other laboratory tests should be requested to aid in the diagnosis?

Thick and thin blood smears

Which of the following is not a factor to be evaluated in the interpretation of a bone marrow aspirate smear?

Type and amount of hemoglobin THESE ARE Maturation of red and white blood cell series b. M:E ratio Estimate of bone marrow activity

Burkitt's lymphoma is histologically characterized by which of the following:

Uniform nuclei b. High mitotic rate c. "Starry-sky" pattern

What are the features of cold agglutinin syndrome?

Usually an IgM antibody b. Reticulocytosis and positive DAT result c. Tendency for spontaneous autoagglutination of RBC samples

All of the following are helpful morphological abnormalities in the diagnosis of MDS EXCEPT:

Vacuolated eosinophils THESE ARE Pseudo Pelger-Huet and hypogranulation Ringed sideroblasts Micromegakaryocytes, monolobular megakaryocytes

What are the mechanisms of immune hemolysis?

a. IgG or IgM antibodies that activate the classic complement cascade b. Antibody-dependent cellular cytoxicity (ADCC) mediated by natural-killer (NK) cells, monocytes/macrophages, and granulocytes c. Complement and phagocytic cells in a reaction of immune adherence

Which of-the following conditions show (s} reactive lymphocytes?

a. Infectious mononucleosis b. Cytomegalovirus c. Hepatitis B

All of the following are clinical manifestations of both vitamin Bl2 and folate deficiency EXCEPT:

d. Hemoglobinuria THESE ARE Anemia and jaundice b. Weakness and shortness of breath c. Thrombocytopenia and bleeding