hematology final

Which of the following conditions is associated with an increase in reticulocyte count?

G6PD deficiency

"Bite cells" are seen in:

G6PD deficiency and unstable hemoglobin variants

Deficiency of membrane____renders PNH erythrocytes susceptible to lysis by activation of ?

GPI-anchored proteins

In hemoglobin C disease, the majority of the red cells appear as ___ on patient with what disease?

target cells

The protein responsible for transporting iron in the bloodstream is:

transferrin

What are the most common structural chromosomal abnormalities in acute lymphoblastic leukemias (ALL) ?

translocations

Which of the following statements of Hodgkin lymphoma is true?

Hodgkin Lymphoma typically spreads from one contiguous lymph node next to the next

which of the following substances are critical for normal hematopoiesis?

-Erythropoietin, colony stimulating factor and thrombopoietin

What is ( are ) the morphological features when viewing the peripheral smear of a severe pernicious anemia?

Pancytopenia, oral shaped macrolytes, and hyperhsegmented neutrophils

Which of the following hemolytic disorders may lead to iron deficiency?

Paroxysmal nocturnal hemoglobinuria

Each day, approximately what percentage of RBC and PLTs in circulatory system of macrophages in the body known as the reticuloendothelial ..?

1% and 10% respectively

Neutropenia is a quantitative disease, generally defined as an absolute neutrophil count (ANC) of less than:

1.5109/L

What is hemoglobin E composed of?

2 alpha and 2 gamma chains

What is the percentage of blasts recommended by WHO classification system for diagnosis of acute leukemia?

20%

What is the composition of normal adult hemoglobin(Hb)?

95-97% HbA;2-3% Hb A2; 1-2% Hb F

The fundamental characteristic of MDS is:

Peripheral blood cytopenia and bone marrow hypercellularity

Causes of bleeding include:

Platelet abnormalities and vWD, Deficency in factor VII, IX, and XI, and Presence of autoantibody against factor VIII or IX

Platelet aggregation is dependent on the presence of:

Platelet glycoprotein IIb/IIIA

Platelet aggregation study is used to assess:

Platelet number

Which leukemia is most commonly encountered in various body organs especially the central nervous system?

ALL

The Ham's test (acidified serum lysis test test) was used in the past for the diagnostic of:

Acquired cold agglutinin disease

A 35- year-old man presents with acute onset of anemia, neutropenia and thrombocytopenia. Blood smear shows numerous "promyelocytes", some with Auer rods.Cytochemistry stain demonstrates that the "promyelocytes" are positive for myeloperoxidase (MPO) and negative for terminal deoxynucleotidyl transferase (TdT). The findings are consistent with which disease?

Acute myeloid leukemia AML

Which of the following is/are indicative of acceleration phase of CML?

Additional chromosomal abnormality , More than 10% bone marrow blasts and Blood basophils increase to >20%

Normal hemoglobin production is dependent upon which mechanism?

Adequate global synthesis, adequate iron supply and normal biosynthesis of protoporphyrins

What is the major inhibitor if thrombin (IIa) and factor Xa?

Antithrombin

The naturally occurring anticoagulants include:

Antithrombin, Protein C and Protein S, and Tissue factor pathway inhibitor

Acquired TTP is most likely caused by:

Autoantibody against ADAMTS13

In the body of an 40 year-old adult, red marrow with active hematopoiesis is mainly found in:

Axial skeleton

Which of the following indicates favorable prognosis in ALL?

B-ALL with 55 chromosomes

The functional defect of which of the following is associated with Southeast Asia?

Band 3

CML can be differentiated from PV, ET, and PMF by presence of:

Bcr-Abl fusion

Which of the following conditions is (are) associated with pancytopenia?

Bone marrow involvement by cancer, B12 or folate deficiency and Aplastic anemia with or without a PNH clone

Which of the following is a function of plasmin?

Breakdown of fibrin

With respect to globin chain genes, which of the following statements is correct?

The genes for the gamma,delta and beta chains are located on chromosome 16 and the genes for the alpha chains are located on chromosome 11

When acute leukemia is suspected in a patient,what laboratory test should be included in the initial diagnostic evaluation?

CBC, with WBC differential and blood smear review, Cytochemical stains and/or flow cytometry immunophenotyping and Chromosome analysis ( also known as cytogenetics)

Follicular Lymphoma is a germinal center B-cell derived from Non-Hodgkin Lymphoma, and is therefore typically positive for

CD10

Which of the following immunotype marker panels should be positive in acute leukemia with Auer rods seen in leukemia rods?

CD13, CD33, CD34

Myeloma cells are usually positive for:

CD79, CD38, CD138

Pyruvate Kinase deficiency is associated with:

Chronic, nonspherocytic hemolysis

Which of the following lab tests may help DIC from TTP?

Coagulation testing shows prolonged PT, PTT, and decreased fibrinogen

All of the following are useful for predicting prognosis for patients with the MDS?

Cytogenetic findings, Prior history of radiation or alkylating agent chemotherapy and Percentage of CD34-positive blast cells in the bone marrow

In patients with Multiple Myeloma, bone destruction and bone lytic lesions with resulting bone pain and pathologic features is caused by which mechanism?

Osteoclast activation

What myeloproliferative neoplasm is characterized by fibrosis in the marrow, extramedullary hematopoiesis in the spleen, leukoerythroblastosis, and tear-drop poikilocytes on blood smear?

PMF

Which of the following can shift the hemoglobin-oxygen dissociation curve to the left

Decrease in 2,3- DPG

The therapeutic effect of warfarin is due to:

Decrease in factors II, VII, IX, X

The pathogenesis of megaloblastic anemia is :

Defective DNA synthesis and abnormal nuclear

Bernard-Soulier Syndrome and glanzmann's thrombasthenia are caused by:

Deficiencies in platelet glycoproteins Ib/IX and IIb/IIIa

Patient's lab testing revealed prolongation of both aPTT and PT. The likely cause is:

Deficiency in Factor X or fibrinogen

Presence of >15% ring sideroblasts (RS) in the bone marrow

Deficient iron utilization

D-Dimers are:

Degradation products of fibrin clot

Increase in fibrinolytic activity leads to:

Delayed bleeding after invasive procedure

Which of the following is not a mechanism of drug-induced immune hemolysis?

Development of alloantibodies against red cells

Which of the following inherited conditions is associated with pure red cell aplasia?

Diamond-Blackfan anemia

Presence of schistocytes from a patient with newly diagnosed acute promyelocytic leukemia is suggestive of which pathologic complication?

Disseminated intravascular coagulation

Schistocytes are seen in which of the following conditions?

Disseminated intravascular coagulation

Patients on long term oral warfarin therapy for atrial fibrillation should be closely monitored with:

PT/INR

Which of the following myeloproliferative disorders can progress to either AML or ALL?

PV

A 16 year-old black high school student is referred by primary care physician to a hematologist for evaluation of acute onset of anemia \.A repeat CBC shows hemoglobin 9.7 g/dL. WBC 9.4103/uL, PLTs 292103/uL, and a reticulocyte count of 295103/uL. Blood smear shows increase in polychromasia and scattered "bite cells", few bands, but no immature cells or blasts.Upon further inquiry, he had a fever and cough a week ago, and his parents worried about pneumonia and gave him "antibiotics" found in the kitchen cabinets.

G6PD enzyme activity should be sent immediately.A normal result rules out G6PD deficiency

Germinal center B-cell derived lymphomas include all of the following:

Follicular lymphoma, Burkitt Lymphoma and Diffuse large B-Cell lymphoma

Vitamin K dependent coagulation factors and hemostasis regulators include:

Factor II and VII, Protein C and protein S , and Factors IX and X

Christmas factor in the coagulation cascade is:

Factor IX

Resistance to activated protein C is associated with:

Factor V Leiden

-Deficiency in which of the following factors does NOT lead to prolongation of activated partial thromboplastin time (aPTT):

Factor VII

Which of the following is (are) used to assess the sufficiency (quality & quantity) of vWF factor?

Factor VIII activity

The cross-linking of fibrin monomers lead to the formation of a fibrin clot. The step is dependent upon:

Factor XIII

The primary targets of activated protein C are:

Factors Va and VIIIa

The RBCs generate their energy (ATP) almost exclusively through which mechanism?

Glycolytic pathway

Platelet adhesion to injured vessel is dependent upon:

Glycoprotein Ib/Ix and von Willebrand factor

In a case of hemolytic anemia with normal bone marrow response, which of the following sets is the expected CBC profile?

Hb,decreased;RBC,decreased;MCV,normal to increased;MCH,normal to increased;RDW,increased.

Which of the following descriptions on extravascular versus intravascular hemolysis in various is NOT correct?

Hemolytic disease of the newborn - intravascular HDN

The principle regulator of body iron metabolism is:

Hepcidin produced by the liver

An osmotic fragility test is performed on a patient's sample.Initial hemolysis solution, and complete hemolysis occurs at 0.50% NaCl.These results correspond to which of the following disorders?

Hereditary spherocytosis

The hemoglobin variant that is seen frequently in the Southeast Asia with ahgb C on alkaline Hgb electrophoresis (pH 8.6) is :

Hgb E

Compare to people living at sea level, a person moves to and lives at high reaching elevations as high as 10,000 feet has a hemoglobin value that is :

Higher

What are the mechanism of immune hemolytic anemia?

IgM antibodies that activate the complement pathway, Phagocytosis of IgM antibody-coated red cells and Phagocytosis of C3b-coated red cells

What blood smear findings are associated with severe myelofibrosis?

Immature granulocytes, nucleated red cells, and tear-drop cells

What is the main mechanism for anemia in patients with chronic inflammatory diseases?

Impairment of storage iron mobilization mediated with hepcidin

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

Imprecision in the G6PD enzyme activity assay

Aspirin inhibits platelets by:

Inactivating platelet COX

A patient has increased cell count, Hgb, and Hct%. Which of following features differentiates secondary polycythemia from polycythemia vera (PV)?

Increase in blood erythropoietin level

18 year old freshman has had nose bleed for over an hour. Has had frequent nose bleeding and skin bruising easily since childhood, along with heavy menstruation since her menarche. She had a prolonged bleeding time. What does this mean, most likely?

Inherited platelet disorder or vWD

Which of the following statements on HIT is (are) correct?

Its associated with thrombosis, Heparin therapy needs to be stopped immediately , and It is associated with platelet activation

The presence of what genetic mutation is one of the major criteria for diagnosis of polycythemia vera (PV)?

Jak2 V617F

A term describing a peripheral blood finding of leukocytosis with a shift to the left (immature granulocytes present), accompanied by presence of nucleated red cells and tear-drop cells is:

Leukoerythroblastosis

Iron-deficiency anemia is characterized by:

Low serum iron, low ferritin, low transferring saturation and high TIBC

Plasma cell neoplasms include:

MGUS, Multiple myeloma and Primary amyloidosis

What causes hemolytic disease of the newborn?

Material IgG antibodies, formed as a result of a previous pregnancy,cross the placenta and attach to fetal red cells

Which qualitative white blood cell (WBC) disorder demonstrates blue-staining cytoplasmic inclusions in neutrophils that resemble Dohle bodies?

May-Hegglin anomaly

MAHA is a result of:

Mechanical fragmentation of red cells in vivo

What specific features are characteristic of morphology id MDS?

Megakaryocytes that are very small and exhibit widely spread nuclei

The anemia of thalassemia intermediate is morphologically characterized as ___________anemia.

Microcytic/hypochromic

Lymphoblastic lymphoma may be associated with:

Monoclonal IgM

Inherited chronic granulomatous disease (CGD) is caused by:

Mutation in the NADPH oxidase

which of the following organisms is (are) associated with hemolytic anemia?

Mycoplasma pneumoniae, Plasmodium falciparum and babesia microti

Oxygen-dependent killing of bacteria by neutrophils is dependent on which enzyme(s)?

NADPH oxidase and myeloperoxidase

Of the following lymphoma types, which is the most commonly seen?

NHL of B-cell type

What findings distinguishes megaloblastic anemia due to vitamin B12deficiency from folate deficiency?

Neuropathy

Describe morphological appearance of the red cells given the following results: RBC: 1.98x10e12/L, Hgb: 7.5g/dL, Hct: 22.0%, RDW: 18.5% (calculate MCV)

Normocytic with anisocytosis

Classical Hodgkin Lymphoma is characterized by the presence of :

Occasional large Reed-Sternberg cells in a background of polymorphic, inflammatory cells

Which would best distinguish hemolytic anemia from other nonimmune hemolytic anemia?

Positive DAT ( direct antiglobulin test)

Which would best distinguish hemolytic anemia caused by immune mechanisms from other nonimmune hemolytic anemia?

Positive DAT (direct antiglobulin test)

Plasma cell myeloma is characterized by:

Presence of monoclonal immunoglobulin in serum

Marked elevation of plasma D-Dimer levels indicates:

Presence of thromboembolism

Assuming no poor cytogenetic abnormality, which of following MDS entities is considered intermediate grade with average survival 2-3 years?

RCMD

The most characteristic finding that may be seen in blood smear of a patient with multiple myeloma and paraprotein is the presence of:

Rouleaux formation

Which of the following statements on vitamin B12 versus folate deficiencies is true?

Serum LDH and indirect bilirubin levels are increased in both conditions

Which membrane protein is principally responsible for formation of inner side of red blood cell membrane?

Spectrins

The function(s) of vW factor is (are):

Surface bound vWF mediates platelet adhesion and Soluble vWF carries coagulation factor VIII

111-The presence of 'CRAB' separates:

Symptomatic myeloma from MGUS and asymptomating (smoldering) myeloma

Reactive lymphocytes seen in blood smear of a young patient diagnosed with infectious mononucleosis represent:

T lymphocytes responding to EBV infection

14 year old male presents with a mediastinal mass, a WBC of 1000x10^3/mcl, hepatosplenomegaly, and central nervous system involvement. Cells express markers CD3, CD4, CD5, CD7, and CD8. What is the diagnosis?

T-ALL

72 year old male was discovered to have progressively decreased Hgb level and neutrophil count since 2 years ago. He turned out to have MDS. With regard to the nature of MDS, which of the following statements is false?

There is a risk of disease progression to acute lymphoblastic leukemia

Where does T-cell differentiation/maturation take place?

Thymus

Of the different types of vWD, which is due to an inherited functional defect in vWF?

Type 2 vWD

The correct sequence of hemostasis after injury is:

Vessel constriction, platelet adhesion and aggregation, activation of coagulation cascade, fibrinolysis

Platelet alpha granules contain:

Von Willebrand Factor

Anemia of chronic disease also known as anemia of chronic inflammation typically associated with:

elevated serum ferritin

Which of the following lab tests is used for monitoring UFH therapy?

aPTT

Which of the following is correct about hemoglobin SC disease?

absence of hemoglobin A on hemoglobin electrophoresis

Hemoglobin H is present in red cells of patients with:

alpha thalassemia

The most common defect seen in hereditary spherocytosis is:

ankyrin deficiency

Switch from mostly hemoglobin F to mostly hemoglobin A occur:

shortly after birth

What disease state can be confirmed by hemoglobin electrophoresis?

sickle cell anemia

Inherited red cell membrane cation permeability alteration causes:

stomatocytosis

Mantle Cell Lymphoma is associated with:

t(11;14)/Cyclin-D1 arrangement

Hgb electrophoresis of 1-year old anemic patient showed migrating bands at A2 and F positions on cellulose acetate gel (alkaline 8.4). Quantitative studies showed 5% A2 and 95% F. What thalassemia is most likely present?

beta thalassemia

Which sequence reflects the correct order for phagocytosis?

binding of particle,ingestion,formation of phagolysosome,Killing and digestion

What feature of RBC morphology is suggestive of Hgb denaturation caused by increased intracellular oxidants?

bite cells

In CML, transformation to a more critical phase is evident by the increase of which cell type in the peripheral blood?

blasts

A rare autosomal recessive congenital disorder that features partial albinism (complete or partial absence of pigments in the skin,hair and eyes), mild bleeding tendencies, and giant lysosomal granules in blood and tissue cells in known as:

chediak-higashi

Which translocation and associated abnormal fusion gene formation are exclusively seen in APML?

t(15;17)/PML-RAR(alpha)

Which of the following can trigger painful crisis in a person with sickle cell disease?

dehydration, hypoxia and infection

Morphological features that can be manifested in the neutrophils in the peripheral blood of patients with septic infections include:

dole bodies, cytoplasmic vacuolization and toxic granulation

Which of the following is characteristic of hereditary spherocytosis (HS)?

extravascular hemolysis

In the coagulation cascade, the intrinsic and extrinsic pathways converge at:

factor X

Sickle cell disease if best differentiated from sickle cell trait by ?

hemoglobin electrophoresis

which of the following is correct about hereditary spherocytosis?

increase in reticulocytes., increase in MCHC and risk for aplastic crisis if infected with parvovirus B19

The main concerns regarding patients with thalassemia major treated with repeated transfusion include(s):

iron overload, alloimmunization and transfusion-transmitted infections disease

Which organ(s) is (are) the site(s) for the hematopoiesis in the fetus?

liver, spleen and bone marrow

Assuming there is no underlying genetic abnormality, presence of which of the following chromosomal abnormalities in AML is associated with more favorable outcome?

t(8;21), Inv (16) and t(15;17)

The key components of the fibrinolytic pathways are:

t-PA and plasminogen

What lab best best indicates EARLY bone marrow response to join iron supplementation therapy in a patient with prolonged iron deficiency anemia?

reticulocyte count

Which of the following is typically associated with mucous membrane and skin bleeding?

vWD

In hemoglobin S and C diseases,___ and___ are substituted for glutamic acid in the 6th position from the N-terminal end of the beta chain, respectively.?

valine and lysine