Hematology Flashcards
How does GPIb become activated in vivo and in vitro, respectively?
shear force, ristocetin
ristocetin, compression
activation of ADP receptor, ristocetin
binding vWF, epinephrine
shear force, ristocetin
The M:E ratio in polycythemia vera is usually:
4:1
10:1
50:1
75:15
4:1
Alloantibodies are found in:
congenital hemophilia
acquired hemophilia
congenital von Willebrand disease
acquired von Willebrand disease
congenital hemophilia
A pediatric cardiac patient presents with a prolonged PT= 18.1 sec (11.5-13.5 sec) and an abnormal aPTT = 51 sec (25-35 sec) with a normal platelet count. Symptoms include bruising and nosebleeds. This is the patients third cardiac surgery, and previously all coagulation results have been normal. A mixing study does not correct for both the PT and aPTT. What should be considered next?
- lupus anticoagulant
- acquired factor inhibitor
-dysfibrinogenemia - DIC evaluation
acquired factor inhibitor
A patient with a clot presents with the results shown in this table:
DIC
hypofibrinogenemia
dysfibrinogenemia
afibrinogenemia
dysfibrinogenemia
Patients with chronic granulomatous disease suffer from frequent pyogenic infections due to the inability of:
lymphocytes to produce bacterial antibodies
eosinophils to degranulate in the presence of bacteria
neutrophils to kill phagocytized bacteria
basophils to release histamine in the presence of bacteria
neutrophils to kill phagocytized bacteria
The type of vWD that has enhanced ristocetin activity is:
type 2A
type 2B
type 2M
type 2N
type 2B
The disease most closely associated with cytoplasmic granule fusion is:
Chédiak-Higashi syndrome
Pelger-Huët anomaly
May-Hegglin anomaly
Alder-Reilly anomaly
Chédiak-Higashi syndrome
A 60-year-old man has a painful right knee and a slightly enlarged spleen. Hematology results include:
This is most compatible with:
congenital spherocytosis
rheumatoid arthritis with reactive thrombocytosis
myelofibrosis
idiopathic thrombocythemia (essential or primary)
idiopathic thrombocythemia (essential or primary)
A useful chemical test for the diagnosis of hairy cell leukemia is the:
peroxidase test
Sudan black test
periodic acid-Schiff test
tartrate-resistant acid phosphatase test
tartrate-resistant acid phosphatase test (TRAP)
The presence of HbH may be demonstrated by:
Prussian blue stain
Wright stain
Giemsa stain
brilliant cresyl blue
brilliant cresyl blue
A patient has been taking aspirin regularly for arthritic pain. Which one of the following tests is most likely to be abnormal in this patient?
platelet count
PFA-100
prothrombin time
activated partial thromboplastin time
PFA-100
Chronic lymphocytic leukemia is defined as a(n):
malignancy of the thymus
accumulation of prolymphocytes
accumulation of hairy cells in the spleen
accumulation of monoclonal B cells
accumulation of monoclonal B cells
What is the most common cause of inherited thrombophilia?
antiphospholipid syndrome
prothrombin G20210A mutation
factor V Leiden
hyperhomocysteinemia
factor V Leiden
Which of the RBC indices is a measure of the amount of hemoglobin in individual red blood cells?
MCHC
MCV
HCT
MCH
MCH
A patient has been treated for a malignant tumor for several years. His blood smear now shows:
-oval macrocytes
-Howell-Jolly bodies
h-ypersegmented neutrophils
-large, agranular platelets
The most probable cause of this blood picture is:
- iron deficiency
- alcoholism
- dietary B12 deficiency
- chemotherapy
chemotherapy
A 65-year-old multiple myeloma patient presents with bruising and hematuria. PT is normal and aPTT and thrombin time are prolonged and do not correct with a mixing study. Fibrinogen and reptilase times are normal. This may be caused by:
heparin-like anticoagulant
acquired vitamin K deficiency
factor deficiency
factor Inhibitor
heparin-like anticoagulant
In a patient 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?
bone marrow panmyelosis
inv(16) mutation
JAK2 V617F mutation
BCR/ABL1 translocation
JAK2 V617F mutation
Heinz bodies are:
readily identified with polychrome stains
remnants of RNA
closely associated with spherocytes
denatured hemoglobin inclusions
denatured hemoglobin inclusions
The principle of platelet aggregation is based on:
decreased light transmission
increased light transmission
decreased light absorbance
increased light absorbance
increased light transmission
A flag of immature granulocytes (IG) is reported from a hematology analyzer. The next step is to perform a(an):
auto verification
smear review
manual differential
pathology review
manual differential
Which of the following hemoglobinopathies is associated with rod shaped crystals?
HbS
HbC
HbSC
HbD
HbC
This AML with recurrent cytogenetic abnormality often presents with disseminated intravascular coagulation:
inv(3)
t(6;9)
t(8;21)
t(15;17)
t(15;17)
A patient sample has been tested in the routine laboratory with a PT and an aPTT. Six hours later factor assays are ordered on the same sample. Which factor is the most labile?
II
VII
VIII
X
VIII
Occasional spontaneous bleeding may occur in a hemophiliac who is classified as:
acquired
mild
moderate
severe
moderate
In HELLP syndrome patients present with:
normal liver enzymes
elevated platelets
high blood pressure
hemochromatosis
high blood pressure
Disorders of platelet aggregation are found in:
von Willebrand disease
Bernard-Soulier
Glanzmann thrombasthenia
storage pool deficiency
Glanzmann thrombasthenia
In comparison to malignant lymphocytes, reactive lymphocytes:
have a denser nuclear chromatin
have a regular cell shape
have more cytoplasm and more mitochondria
are morphologically more variable throughout the smear
are morphologically more variable throughout the smear
Biological assays for antithrombin (AT) are based on the inhibition of:
factor VIII
heparin
serine proteases
anti-AT globulin
serine proteases
Which of the following is used for staining reticulocytes?
Giemsa stain
Wright stain
new methylene blue
Prussian blue
new methylene blue
Which of the following values is calculated from the red blood cell indices in an automated hematology analzyer?
red blood cell count (RBC)
hematocrit (HCT)
mean corpuscular volume (MCV)
red cell distribution width (RDW)
hematocrit (HCT)
Which of the following statements about hemoglobins D and G is true?
they are clinically abnormal
they both migrate with HbS on alkaline gel
they are both caused by mutations in the beta-globin gene
they cannot be separated by solubility testing
they both migrate with HbS on alkaline gel
Biochemical abnormalities characteristic of polycythemia vera include:
increased serum B12 binding capacity
hypouricemia
hypohistaminemia
increased erythropoietin
increased erythropoietin
Which of the following is a WHO classification of myelodysplastic syndrome?
chronic myelomonocytic leukemia (CMML)
MDS with low blasts
refractory anemia with excessive blasts (RAEB)
refractory neutrophilia
MDS with low blasts
Which substrate is used for the detection of specific esterase?
acetate
chloroacetate
pararosanilin acetate
phenylene diacetate
chloroacetate
A bone marrow shows foam cells ranging from 20 - 100 µm, vacuolated cytoplasm containing sphingomyelin and faint PAS positivity. This cell type is most characteristic of:
Gaucher disease
myeloma with Russell bodies
DiGuglielmo disease
Niemann-Pick disease
Niemann-Pick disease
Which of the following are found in association with megaloblastic anemia?
neutropenia and thrombocytopenia
decreased LD activity
increased erythrocyte folate levels
decreased plasma bilirubin levels
neutropenia and thrombocytopenia
A 30-year-old man who had been diagnosed as having leukemia 2 years previously is readmitted because of cervical lymphadenopathy. Laboratory findings are shown in this table:
These results are most consistent with:
acute myeloid leukemia
erythroleukemia
chronic myelogenous leukemia (CML)
CML in blast transformation
CML in blast transformation
A 56-year-old man is admitted to the hospital for treatment of a bleeding ulcer. The laboratory data shown in this table are obtained: Examination of the bone marrow reveals the absence of iron stores. This data is most consistent with which of these conditions?
iron deficiency anemia
anemia of chronic inflammation
hemochromatosis
acute blood loss
iron deficiency anemia
The calculated erythrocyte indices on an adult man are MCV = 89 fL, MCH = 29 pg and MCHC = 38%. The calculations have been rechecked; erythrocytes on the peripheral blood smear appear normocytic and normochromic with no abnormal forms. The next step is to:
report the results
examine another smear
repeat the hemoglobin and hematocrit
repeat the erythrocyte count and hematocrit
repeat the hemoglobin and hematocrit
Heparin induced thrombocytopenia (HIT) is an immune mediated complication associated with heparin therapy. Antibodies are produced against:
ACLA
PF4
AT
B2GP1
PF4
The disease most frequently present in patients with reactive lymphocytosis and persistently negative heterophile antibody tests is:
toxoplasmosis
cytomegalovirus (CMV) infection
herpes virus infection
viral hepatitis
cytomegalovirus (CMV) infection
A newborn baby boy is known to have a homozygous protein C deficiency. This puts him at a risk for:
DVT
warfarin induced skin necrosis
increased risk of thrombosis
purpura fulminans
purpura fulminans
One of the major glands in an infant primarily responsible for producing lymphocytes is the:
thymus
adrenal
thyroid
pituitary
thymus
The major serine protease responsible for clot breakdown is:
TPA
alpha-2 antiplasmin
streptokinase
PAI-1
TPA
Which of the following cytokines is most responsible for eosinophil differentiation and release from the bone marrow?
IL-1
IL-2
IL-4
IL-5
IL-5
Which of the following is most closely associated with acute promyelocytic leukemia?
ringed sideroblasts
disseminated intravascular coagulation
micromegakaryocytes
Philadelphia chromosome
disseminated intravascular coagulation
Hemoglobin H disease results from:
absence of 3 of 4 alpha genes
absence of 2 of 4 alpha genes
absence of 1 of 1 alpha genes
absence of all 4 alpha genes
absence of 3 of 4 alpha genes
The cell in the center of the photomicrograph is a:
Promyelocyte
lymphocyte
neutrophil
monocyte
Monocyte (beware there are punched out vacuoles AND red dots on this…it doesn’t look like a mono to me)
Given the data in this table, what is the absolute lymphocyte count?
Table:
WBC 8.5 x 10^3 uL
Neutrophils 56%
Bands 2%
Lymphs 30%
Monos 6%
Eos. 6%
170/µL (0.17 × 109/L)
510/µL (0.51 × 109/L)
2550/µL (2.55 × 109/L)
4760/µL (4.76 × 109/L)
2550/µL (2.55 × 109/L)
In most cases of hereditary persistence of fetal hemoglobin (HPFH):
hemoglobin F is unevenly distributed throughout the erythrocytes
the black heterozygote has 75% hemoglobin F
beta- and gamma-chain synthesis is decreased
gamma-chain production equals alpha-chain production
gamma-chain production equals alpha-chain production
What cell shape is most commonly associated with an increased MCHC?
teardrop cells
target cells
spherocytes
sickle cells
spherocytes
Which of the following is most closely associated with idiopathic hemochromatosis?
iron overload in tissue
target cells
Cabot rings
ringed sideroblasts
iron overload in tissue
What is the most common presentation of acute erythroid leukemia?
> 80% of nucleated marrow cells are erythroid precursors
> 50% of nucleated marrow cells are erythroid precursors and >20% of nonerythroid cells are myeloblasts
> 50% of nucleated marrow cells are erythroid precursors and <20% of nonerythroid cells are myeloblasts
80% blasts with 20% erythroid precursors, >20% myeloblasts
> 80% of nucleated marrow cells are erythroid precursors
Which of the following is the most common cause of anemia in hospitalized patients?
inadequate iron intake
inadequate folate intake
hemolytic anemia
anemia of chronic inflammation
anemia of chronic inflammation
Which of the following tumors are associated with erythrocytosis due to excessive erythropoietin production?
renal cell carcinoma
sarcoma
basal cell carcinoma
squamous cell carcinoma of the lung
renal cell carcinoma
Antithrombin inhibits factors:
IIa and Xa
Va and VIIIa
VIIa and XIIa
IXa and Va
IIa and Xa
The type of bleeding that is the most characteristic in patients with platelet disorders is:
deep muscle hemorrhages
retroperitoneal hemorrhages
mucous membrane hemorrhages
severely prolonged clotting times
mucous membrane hemorrhages
Which abnormal RBC morphology is associated with pyruvate kinase deficiency?
acanthocytes
dacryocytes
echinocytes
drepanocytes
echinocytes
Which of the following will not affect the PFA-100 closure time?
aspirin
thrombocytopenia
anemia
leukopenia
leukopenia
In patients who present with bleeding disorders caused by platelets, the most common type of bleeding is:
mucosal bleeding
hemarthrosis
delayed bleeding
deep hematomas
mucosal bleeding
A differential is performed on an asymptomatic patient. The differential included 60% neutrophils, 55 of which had 2 lobes and 5 had 3 lobes. There are no other abnormalities. This is consistent with which of these anomalies?
Pelger-Huët
May-Hegglin
Alder-Reilly
Chédiak-Higashi
Pelger-Huët
Vasoconstriction is caused by several regulatory molecules, which include:
fibrinogen and vWF
ADP and EPI
thromboxane A2 and serotonin
collagen and actomyosin
thromboxane A2 and serotonin
What subendothelial structural protein triggers coagulation through activation of FVII?
thrombomodulin
nitric oxide
tissue factor
silica
tissue factor
The test used to quantitate a factor inhibitor is the:
factor assay
multimer test
ristocetin cofactor
Bethesda assay
Bethesda assay
A patient that has a lupus anticoagulant may bleed due to:
antibodies to prothrombin
factor VII deficiency
infection
factor VIII deficiency
antibodies to prothrombin
Which of the following platelet responses is most likely associated with type IIb von Willebrand disease?
decreased platelet aggregation to low-dose ristocetin
normal platelet aggregation to low-dose ristocetin
increased aggregation to low-dose ristocetin
decreased aggregation to high-dose ristocetin
increased aggregation to low-dose ristocetin
Whole blood evaluation of blood clotting can be performed by using thromboelastography. This methodology looks at:
clot onset, agglutination, fibrinolysis
clot onset, aggregation, clot strength
aggregation, clot onset, clot strength
clot onset, clot strength, fibrinolysis
clot onset, clot strength, fibrinolysis
Which of the following stains is used to demonstrate iron, ferritin and hemosiderin?
myeloperoxidase
methylene blue
specific esterase
Prussian blue
Prussian blue
Acute disseminated intravascular coagulation is characterized by:
hypofibrinogenemia
thrombocytosis
negative D-dimer
shortened thrombin time
hypofibrinogenemia
von Willebrand factor mediates platelet adhesion by binding to platelet receptor:
GPIb/IIa
GPIb/GPIX/GPV
GPIIb/IIa
GPIb/GPIIIa/GP X
GPIb/GPIX/GPV
A phase-platelet count is performed and the total platelet count is 356,000/µL (3.56 × 1012/L). Ten high power (100x) fields on the stained blood smear are examined for platelets and the results per field were:
16, 18, 15, 20, 19, 17, 19, 18, 20, 16
The next step would be to:
report the phase-platelet count since it correlated well with the slide
repeat the phase-platelet count on a recollected specimen and check for clumping
check 10 additional fields on the blood smear
repeat the platelet count using a different method
report the phase-platelet count since it correlated well with the slide
In the Clauss fibrinogen assay the time for clot formation in plasma is measured after the addition of:
calcium
thrombin
phospholipids
fibrin
thrombin
A 68-year-old male presents with tingling in hands and feet, an irregular heart beat, severe fatigue and difficulty in swallowing. Coagulation results show an abnormal PT and aPTT, which completely correct to normal with a mixing study. The factor most likely that contributes to this is a deficiency in:
factor VII
factor X
factor VIII
factor XIII
factor X
What type of morphologically abnormal cells proliferate in acute myelogenous leukemia with inv(16)?
lymphocytes
erythrocytes
platelets
eosinophils
eosinophils
Peripheral blood smears from patients with untreated pernicious anemia are characterized by:
pancytopenia and macrocytosis
leukocytosis and elliptocytosis
leukocytosis and ovalocytosis
pancytopenia and microcytosis
pancytopenia and macrocytosis
A patient presents with a factor VIII level of 2%. The vWF activity (ristocetin cofactor) is <1% with a vWF antigen of 3%. The most likely diagnosis is:
hemophilia A
hemophilia B
type II vWD
type III vWD
type III vWD
Which of the following markers are usually negative in hairy cell leukemia?
CD5
CD11c
CD25
CD103
CD5
A 20-year-old woman with sickle cell anemia, whose usual hemoglobin concentration
is 8 g/dL (80 g/L), develops fever, increased weakness and malaise. The hemoglobin concentration is 4 g/dL (40 g/L) and the reticulocyte count is 0.1%. The most likely explanation for her clinical picture is:
increased hemolysis due to hypersplenism
aplastic crisis
thrombotic crisis
occult blood loss
aplastic crisis
The most common form of childhood leukemia is:
acute lymphocytic
acute granulocytic
acute monocytic
chronic granulocytic
acute lymphocytic
The white cell feature most characteristic of pernicious anemia is:
eosinophilia
toxic granulation
hypersegmentation
reactive lymphocytes
hypersegmentation
The disease most closely associated with granulocyte hyposegmentation is:
May-Hegglin anomaly
Pelger-Huët anomaly
Chédiak-Higashi syndrome
Gaucher disease
Pelger-Huët anomaly
A patient that has a lupus anticoagulant may bleed due to:
factor VIII deficiency
drugs
antibodies to prothrombin
infection
antibodies to prothrombin
The reactive lymphocyte seen in the peripheral blood smear of patients with infectious mononucleosis is probably derived from which of these cell types?
T lymphocytes
B lymphocytes
monocytes
mast cells
T lymphocytes
Spontaneous bleeding occurs when the platelet count is:
<10 × 10^3/µL
<20 × 10^3/µL
<50 × 10^3/µL
<100 × 10^3/µL
<20 × 10^3/µL
A patient is on unfractionated heparin after surgery and has an aPTT of 65 seconds, however the patient has developed a DVT. The best test to monitor this patient with is:
factor X assay
factor II assay
anti-Xa assay
antithrombin assay
anti-Xa assay
Which of the following is associated with May-Hegglin anomaly?
membrane defect of lysosomes
Döhle bodies and giant platelets
chronic myelogenous leukemia
mucopolysaccharidosis
Döhle bodies and giant platelets
What protein is commonly defective in hereditary elliptocytosis?
ankyrin
spectrin
band 4.1
elliptocin
spectrin
Which of the following cytogenetic abnormalities is associated with a more stable clinical course in myelodysplastic syndromes (MDSs)?
monosomy 7
del 5q
loss of Y
del 20q
del 5q
A 60-year-old female presents with blurred vision, confusion, fever and severe bruising with a platelet count of 29 × 10^3/µL (29 × 10^9/L).
Test results: PT 12.1 sec (range 11.4 - 13.5) and aPTT 32.6 sec (range 25 - 35). The CBC RBC morphology shows schistocytes. The differential diagnosis should include:
ITP
TTP
HUC
DIC
TTP
Which of the following markers is expressed in most cases of AML?
CD2
CD10
CD11b
CD117
CD117
The hemoglobin variant that is seen frequently in the South East Asian population, demonstrates a microcytic blood smear, and migrates with HbC at pH 8.6 is:
HbBarts
HbF
HbE
HbH
HbE
Which of the following RBC inclusions are seen in sideroblastic anemia and contain high amounts of iron?
Cabot rings
Howell-Jolly bodies
Heinz bodies
Pappenheimer bodies
Pappenheimer bodies
A 14-year-old boy is seen in the ER complaining of a sore throat, swollen glands and fatigue. The CBC results are:
What is the most likely diagnosis?
acute lymphocytic leukemia
chronic lymphocytic leukemia
viral hepatitis
infectious mononucleosis
infectious mononucleosis
Given this normal scattergram, the area indicated as a “6” represents which cell population?
monocytes
large unstained cells
platelet clumps
nucleated RBCs
monocytes
The disease state that presents with a quantitative platelet disorder is:
von Willebrand disease
hemophilia A
Glanzmann thrombasthenia
May-Hegglin anomaly
May-Hegglin anomaly
Which of the following is associated with Alder-Reilly inclusions?
membrane defect of lysosomes
Döhle bodies and giant platelets
2-lobed neutrophils
mucopolysaccharidosis
mucopolysaccharidosis
In secondary hemostasis, coagulation proteins become activated to form a fibrin clot. Prior to these proteins being activated, their inactive proenzymes are known as:
serine proteases
cofactors
zymogens
substrates
zymogens
Specific (secondary) granules of the neutrophilic granulocyte:
appear first at the myelocyte stage
contain esterases
are formed on the mitochondria
are derived from azurophil (primary) granules
appear first at the myelocyte stage
A patient has a high cold agglutinin titer. Automated hematology analyzer results reveal an elevated MCV, MCH and MCHC as well as a decreased RBC. Individual erythrocytes appear normal on a stained smear, but agglutinates are noted. The appropriate course of action would be to:
perform the RBC, HGB, and HCT determinations using manual methods
perform the RBC determination by a manual method; use the automated results for the HGB and HCT
repeat the determinations using a microsample of diluted blood
repeat the determinations using a prewarmed microsample of diluted blood
repeat the determinations using a prewarmed microsample of diluted blood
