Hematology Part 1

The majority of the iron in an adult is found as a constituent of:

Hemoglobin

In order for hemoglobin to combine reversibly with oxygen, the iron must be:

in the ferrous state

Which description best fits the Donath-Landsteiner antibody?

IgG biphasic hemolysin

Which of the following ions is bound to hemoglobin in methemoglobin?

Fe3+

An increased amount of cytoplasmic basophilia in a blood cell indicates:

Decreased cytoplasmic maturation

Specific (secondary) granules of neutrophilic granulocyte:

Appear first at the myelocyte stage

In the normal adult bone marrow, the most common granulocyte is the: 

Metamyelocyte 

Pluripotent hematopoietic stem cells are capable of producing: 

Lymphoid and Myeloid Stem Cells

Which of the following cytokines is most responsible for eosinophil differentiation and release from the bone marrow?

IL-5

Auer rods are characterized as: 

Fused primary granules 

Which of the following is characteristic of cellular changes as megakaryoblasts mature into megakaryocytes within the bone marrow?

Nuclear division without cytoplasmic maturation

Which of the following cells is the largest cell in the bone marrow:

Megakaryocyte

After the removal of red blood cells form the circulation hemoglobin is broken down into:

Iron, Heme, Globin

The main function of the hexose monophosphate shunt is the erythrocyte is to:

Provide reduced glutathione to prevent hemoglobin oxidation

In the normal adult, the spleen acts as a site for:

Removal of imperfect and aging cells

Cells for the transport of O2 and CO2 are: 

Erythrocytes 

Erythropoietin acts to:

Stimulate RNA synthesis of erythroid cells

Cells that produce antibodies and are capable of direct cytolysis:

Lymphocytes

Phagocytosis is a function of:

Granulocytes

Which cells are involved in immediate hypersensitivity reactions?

Basophils 

One of the major glands in an infant primarily responsible for producing lymphocytes is the:

Thymus

When iron in hemoglobin is in the +3 state, it is termed:

Methemoglobin

Phagocytosis in neutrophils can be described as a process to:

Kill and degranulate bacteria

The characteristic erythrocyte found in pernicious anemia is: 

Macrocytic 

Hemolysis in paroxysmal nocturnal hemoglobinuria (PNH) is:

Caused by a red cell membrane defect

Which of the following is most closely associated with idiopathic hemochromatosis?

Iron overload in tissue

A pt with polycythemia vera who is treated by phlebotomy is most likely to develop a deficiency of:

Iron

The direct anti globulin test (DAT) can help distinguish: 

Inherited from acquired spherocytosis 

The anemia of chronic inflammation is characterized by:

Decreased serum iron levels

Factors commonly involved in causing anemia in pts with chronic renal disease include:

Inadequate Erythropoiesis

The hypo proliferative red cell population in the bone marrow of uremic pts is caused by:

Decreased levels of circulating erythropoietin

Which of the following characteristics are common to hereditary spherocytosis, elliptocytosis, stomatocytosis, and PNH?

Red Cell Membrane Defects 

Which of the following is most closely associated with IDA?

Chronic Blood Loss

Evidence indicates that the genetic defect in thalassemia usually results in:

A quantitative deficiency in RNA resulting in decreased globin chain production

Anemia secondary to uremia and chronic renal disease characteristically is: 

Normocytic, Normochromic 

Which of the following sets of lab finding is constant with hemolytic anemia?

Increased serum lactate dehydrogenase and catabolism of heme

Deficiency of what enzyme is associated with a moderate to severe hemolytic anemia after the pt is exposed to certain drugs and characterized by red cell inclusions formed by denatured hemoglobin:

G6PD 

Pts with G6PD deficiency are least likely to have hemolytic episodes in which of the following situations?

Spontaneously

A pt has a congenital nonspherocytic hemolytic anemia. After exposure to anti-malarial drugs, the pt experiences a severe hemolytic episode. This episode is characterized by red cell inclusions caused by hemoglobin denaturation, which of the following conditions is most consistent with these findings?

G6PD Deficiency

Which of the following is the most characteristic finding in autoimmune hemolytic anemia?

Positive DAT 

Peripheral blood smears form pts with untreated pernicious anemia are characterized by:

Pancytopenia and Macrocytosis

Lab tests performed on a pt indicate macrocytosis and pancytopenia. Which of the following disorders is most likely?

Vitamin B12 deficiency

The characteristic morphologic feature in folic acid deficiency is:

Macrocytosis

Megaloblastic asynchronous development in the bone marrow indicates which one of the following?

Impaired synthesis of DNA

Which of the following are found in association with megaloblastic anemia?

Neutropenia and Thrombocytopenia 

A characteristic morphologic feature in hemoglobin C disease is:

Target Cells

Thalassemias are characterized by:

Decreased rate of globin synthesis

Lab findings in hereditary spherocytosis include:

Decreased RBC band 3 protein

Which of the following types of polycythemia is a severely burned pt most likely to have?

Relative polycythemia associated with dehydration

The characteristic morphologic feature in lead poisoning is:

Basophilic Stippling 

The white cell feature most characteristic of pernicious anemia is: 

Hypersegmentation

Which parameter is most consistently abnormal in cases of hereditary spherocytosis?

MCHC

What protein is commonly defective in hereditary elliptocytosis?

Spectrin

What is the most common mechanism resulting in hereditary stomatocytosis?

Abnormal Na/K permeability

The basic mechanism associated with the development of sideroblastic anemia is: 

Enzymatic defect in heme synthesis causes iron accumulation 

Which of the following features of G6PD deficiency are typically present on a Wright-Giemsa stained peripheral blood smear?

Bite Cells

Which abnormal RBC morphology is associated with pyruvate kinase deficiency?

Echinocytes

Which of the following hemoglobinopathies is associated with rod shaped crystals?

HbC

Which of the following is consistent with the diagnosis of heterozygous beta-thalassemia?

Increased RBC count

What is the specificity of cold auto agglutinin disease?

Anti-I

What is the most common presentation of paroxysmal cold hemoglobinuria?

Children following a viral illness

Which of the following is the most common cause of anemia in hospitalized pts?

Anemia of chronic inflammaiton

In a pt with an increased red cell mass into the 99th percentile and serum erythropoietin level below reference range for normal, which of the following criteria confirms a diagnosis of polycythemia vera?

JAK2 V617F mutation 

Hemoglobin H disease results from:

Absence of 3 of 4 alpha genes

The M:E ratio in polycythemia vera is usually:

4:1

A pt has a tumor that concentrates erythropoietin. He is most likely to have which of the following types of polycythemia?

Polycythemia associated with renal diseaes

Which of the following types of polycythemia is most often associated with lung disease?

Polycythemia, Secondary to hypoxia

Oval Macrocytes; Howell-Jolly Bodies; Hypersegmented Neutrophils; Large, granular paltelets:

Chemotherapy 

How does the bone marrow respond to anemic stress? 

Expand production, Release RBCs prematurely 

Which of the following conditions may contribute to lethargy, abdominal pain, and hemoglobinuria in some pts with a G6PD deficiency?

Ingesting fava beans 

The most likely cause of the macrocytosis that often accompanies primary myelofibrosis is:

Folic Acid Deficiency

Giant, Vacuolated, Multinucleated erythroid precursors are present in which of the following?

Erythroleukemia

Which of the following is a significant feature of dyserythropoiesis?

Megaloblastoid Erythropoiesis

The M:E ratio in erythroleukemia is usually:

Low

Autoimmune hemolytic anemia is often a complication of:

CLL

Elevation of the total granulocyte count above 7.5×10³/uL is termed:

Absolute neutrophilic leukocytosis

Elevation of the total white cell count above 11.0×10³/uL is termed:

Leukocytosis

Elevation of the lymphocyte percentage above 45% is termed:

Relative Lymphocytosis

The Philadelphia chromosome is formed by a translocations between:

22 and 9

What accounts for the frequent smudge cells in CLL?

Artifact due to fragile cells

The M:E ratio in chronic myelocytic leukemia is usually:

High

In the World Health Organization classification, acute myelomonocytic leukemia would be named acute myeloid leukemia (AML)

Not other wise specific

Abnormalities found in erythroleukemia include:

Megaloblastoid development

Neutropenia is usually associated with:

Viral Infections

Auer rods are most likely present tin which of the following?

Acute Myelocytic Leukemia

In an uncomplicated case of infectious mono, which type of cells is affected?

Lymphocytes

The reactive lymphocyte seen in the peripheral blood smear of patients with infectious mono is probably derived form which of these cell types?

T lymphocytes

The disease most frequently present in pts with reactive lymphocytosis and persistently negative heterophile antibody tests is:

CMV

Dwarf or micromegakaryocytes may be found in the blood of pts with:

Primary Meylofibrosis

Which of the following is associated with pseudo-pelger-huett anomaly?

CML

Increased numbers of basophils are often seen in:

CML

Biochemical abnormalities characters of polycythemia vera include:

increased B12 binding capacity

A diff cell count of 50-90% myeloblasts in a blood smear is indicative of:

AML

The M:E ratio in AML is usually:

High

Which of the following is most closely associated with acute promyelocytic leukemia?

DIC

Which fo the following is most closely associated with CML?

Lysozymuria

Which of the following is most closely associated with CML?

BCR::ABL 1 fusion gene

The replacement of normal marrow precursor cells by an accumulation of blasts is a hallmark of:

AML

All stages of neutrophils are most likely to be seen in the blood of a pt with:

CML

Which of the following conditions is a myeloproliferative neoplasm?

Essential Thrmobocythemia