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Which of the following inherited leukocyte disorders is caused by a mutation in the lamin B receptor?
Pelger-Huet Anomaly
Chediak-Higashi disease
Alder-Reilly anomaly
May-Hegglin anomaly
Pelger-Huet Anomaly
Which of the following inherited leukocyte disorders involves mutations in nonmuscle myosin heavy-chain IIA?
Pelger-Huet Anomaly
Chediak-Higashi disease
Alder-Reilly anomaly
May-Hegglin anomaly
May-Hegglin anomaly
Which of the following inherited leukocyte disorders might be seen in Hurler syndrome?
Pelger-Huet anomaly
Chediak-Higashi disease
Alder-Reilly anomaly
May-Hegglin anomaly
Alder-Reilly anomaly
Which of the following lysosomal storage diseases is characterized by macrophages with striated cytoplasm and storage of glucocerebroside?
Sanfilippo syndrome
Gaucher disease
Fabry disease
Niemann-Pick disease
Gaucher disease
The neutrophils in chronic granulomatous disease are incapable of producing:
Hydrogen peroxide
Hypochlorite
Superoxide
All of the above
All of the above
Individuals with X-linked SCID have a mutation that affects their ability to synthesize:
Deaminase
Oxidase
IL-2 receptor
IL-8 receptor
IL-2 receptor
An absolute lymphocytosis with reactive lymphocytes suggests which of the following conditions?
DiGeorge syndrome
Bacterial infection
Parasitic infection
Viral infection
Viral infection
What leukocyte cytoplasmic inclusion is composed of ribosomal RNA?
Primary granules
Toxic granules
Dohle bodies
Howell-Jolly bodies
Dohle bodies
The expected complete blood cell count (CBC) results for women in active labor would include:
High total white blood cell (WBC) count with increased lymphocytes
High total WBC count with a slight shift to the left in neutrophils
Normal WBC count with increased eosinophils
Low WBC count with increased monocytes
High total WBC count with a slight shift to the left in neutrophils
Which of the following is true of an absolute increase in lymphocytes with reactive morphology?
The population of lymphocytes appears morphologically homogenous
They are usually effector B cells
The reactive lymphocytes have increased cytoplasm with variable basophilia
They are most commonly seen in bacterial infections
The reactive lymphocytes have increased cytoplasm with variable basophilia
According to the WHO classification, except in leukemias with specific genetic anomalies, the minimal percentage of blasts necessary for a diagnosis of acute leukemia is:
10%
20%
30%
50%
20%
A 20-year-old patient has an elevated WBC count with 70% blasts, 4% neutrophils, 5% lymphocytes, and 21% monocytes in the peripheral blood. Eosinophils with dysplastic changes are seen in the bone marrow. AML with which of the following karyotypes would be most likely to be seen?
AML with t(8;21)(q22;q22)
AML with t(16;16)(p13;q22)
APL with PML-RARA
AML with t(9;11)(p22;q23)
AML with t(16;16)(p13;q22)
Which of the following would be considered a sign of potentially favorable prognosis in children with ALL?
Hyperdiploidy
Presence of CD19 and CD20
Absence of trisomy 8
Presence of BCR/ABL gene
Hyperdiploidy
Signs and symptoms of cerebral infiltration with blasts are more commonly seen in:
AML with recurrent cytogenetic abnormalities
Therapy-related myeloid neoplasms
AML with myelodysplasia-related changes
ALL
ALL
An oncology patient exhibiting signs of renal failure with seizures after initial chemotherapy may potentially develop:
Hyperleukocytosis
Tumor lysis syndrome
Acute leukemia secondary to chemotherapy
Myelodysplasia
Tumor lysis syndrome
Disseminated intravascular coagulation is more often seen in association with leukemia characterized by which of the following mutations?
t(12,21)(p13;q22)
t(9;22)(q34;q11.2)
inv(16)(p13;q22)
t(15;17)(q22;q12)
t(15;17)(q22;q12)
Which of the following leukemia affects primarily children, is characterized by an increase in monoblasts and monocytes, and often is associated with gingival and skin involvement?
Pre-B lymphoblastic leukemia
Pure erythroid leukemia
AML with t(9;11)(p22;q13)
APL with PML-RARA
AML with t(9;11)(p22;q13)
A 20-year-old patient present with fatigue, pallor, easy bruising, and swollen. Bone marrow examination reveals 82% cells with delicate chromatin and prominent nucleoli that are CD14+, CD4+, CD11b+, and CD36+.
Minimally differentiated leukemia
Leukemia of ambiguous lineage
Acute monoblastic/monocytic leukemia
Acute megakaryoblastic leukemia
Acute monoblastic/monocytic leukemia
Pure erythroid leukemia is a disorder involving:
Pronormoblasts only
Pronormoblasts and basophilic normoblasts
All forms of developing RBC precursors
Equal number of pronormoblasts and myeloblasts
Pronormoblasts and basophilic normoblasts
A patient with normal chromosomes has a WBC count of 3.0 x 109/L and dysplasia in all cell lines. There are 60% blasts of varying sizes. The blasts stain positive for CD61. The most likely type of leukemia is:
Acute lymphoblastic
Acute megakaryoblastic
Acute monoblastic
APL with PML-RARA
Acute megakaryoblastic
SBB stains which of the following component of cells?
Glycogen
Lipids
Structural proteins
Enzymes
Lipids
The cytochemical stain ɑ-napthyl butyrate is a nonspecific esterase stain that shows diffuse positivity in cells of which lineage?
Erythroid
Monocytic
Granulocytic
Lymphoid
Monocytic
A peripheral blood film that shows increased neutrophils, basophils, eosinophils, and platelets is highly suggestive of:
AML
CML
MDS
Multiple myeloma
CML
Which of the following chromosome abnormalities is associated with CML?
t(15;17)
t(8;14)
t(9;22)
Monosomy 7
t(9;22)
A patient has a WBC count of 30 x 109/L and the following WBC differential:
Segmented neutrophils – 38%
Bands — 17%
Metamyelocytes — 7%
Myelocytes — 20%
Promyelocytes — 10%
Eosinophils — 3%
Basophils — 5%
Which of the following test results would be helpful in determining whether the patient has CML?
Nitroblue tetrazolium reduction product increased
Myeloperoxidase increased
Periodic acid–Schiff staining decreased
FISH positive for BCR-ABL1 fusion
FISH positive for BCR-ABL1 fusion
A patient in whom CML has previously been diagnosed has circulating blasts and promyelocytes that total 30% of leukocytes. The disease is considered to be in what phase?
Chronic stable phase
Accelerated phase
Transformation to acute leukemia
Temporary remission
Transformation to acute leukemia
The most common mutation found in patients with primary PV is:
BCR-ABL1
Philadelphia chromosome
JAK2 V617F
t(15;17)
JAK2 V617F
The peripheral blood in PV typically manifests:
Erythrocytosis only
Erythrocytosis and thrombocytopenia
Erythrocytosis, thrombocytosis, and granulocytosis
Anemia and thrombocytopenia
Erythrocytosis, thrombocytosis, and granulocytosis
A patient has a platelet count of 700 x 109/L with abnormalities in the size, shape, and granularity of platelets; a WBC count of 12 x 109/L; and hemoglobin of 11 g/dL. The Philadelphia chromosome is not present. The most likely diagnosis is:
PV
ET
CML
Leukemoid reaction
ET
Complications of ET include all of the following except:
Thrombosis
Hemorrhage
Seizures
Infections
Infections
Which of the following patterns is characteristic of the peripheral blood in patients with PMF?
Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes
Abnormal platelets only
Hypochromic erythrocytes, immature granulocytes, and normal platelets
Spherocytes, immature granulocytes, and increased numbers of platelets
Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes
The myelofibrosis associated with PMF is a result of:
Apoptosis resistance in the fibroblasts of the bone marrow
Impaired production of normal collagenase by the mutated cells
Enhanced activity of fibroblasts as a result of increased stimulatory cytokines
Increased numbers of fibroblasts as a result of cytokine stimulation of the pluripotential stem cells
Enhanced activity of fibroblasts as a result of increased stimulatory cytokines
MDS are most common in which age group?
2 to 10 years
15 to 20 years
25 to 40 years
Older than 50 years
Older than 50 years
What is a major indication of MDS in the peripheral blood and bone marrow?
Dyspoiesis
Leukocytosis with left shift
Normal bone marrow with abnormal peripheral blood features
Thrombocytosis
Dyspoiesis
An alert hematologist should recognize all of the following peripheral blood abnormalities as diagnostic clues in MDS EXCEPT:
Oval macrocytes
Target cells
Agranular neutrophils
Circulating micromegakaryocytes
Target cells
For an erythroid precursor to be considered a ring sideroblast, the iron-laden mitochondria must encircle how much of the nucleus?
One-quarter
One-third
Two-thirds
Entire nucleus
One-third
According to the WHO classification of MDS, what percentage of blasts would constitute transformation to an acute leukemia?
5%
10%
20%
30%
20%
A patient has anemia, oval macrocytes, and hypersegmented neutrophils. Which of the following tests would be most efficient in differential diagnosis of this disorder?
Serum iron and ferritin levels
Erythropoietin level
Vitamin B12 and folate levels
Chromosome analysis
Vitamin B12 and folate levels
Into what other hematologic disease does MDS often convert?
Megaloblastic anemia
Aplastic anemia
AML
Myeloproliferative disease
AML
Chronic myelomonocytic leukemia is classified in the WHO system as:
A myeloproliferative neoplasm
Myelodysplastic syndrome, unclassified
MDS/MPN
Acute leukemia
MDS/MPN
Non-Hodgkin lymphoma can be best differentiated from reactive disorders by:
Genetic testing
Immunophenotyping
Absolute lymphocyte count
Blood film review
Blood film review
Which laboratory test is most suggestive of autoimmune hemolytic anemia in a patient with CLL?
Direct antiglobulin test
Hemoglobin
Lymphocyte count
Platelet count
Lymphocyte count
What is the best test or method for determining if a clonal population of T cells is present in a specimen?
Molecular diagnostic testing
Flow cytometry for CD3, CD5, and CD7
Immunohistochemical stain
Karyotyping
Karyotyping
A rise in the lymphocyte count from 4.1 x 109/L to 5.5 x 109/L in a patient with monoclonal B lymphocytosis suggests:
Acute lymphocyte leukemia
Chronic lymphocytic leukemia
Acute myelocytic leukemia
A reactive condition
A reactive condition
Which test is often used to differentiate CLL from mantle cell lymphoma?
Annexin A staining
Lymph node biopsy
Immunohistochemistry
FISH for BCL2 translocation
Lymph node biopsy
If not treated, which of the following would generally be associated with the best outcome?
Peripheral T cell lymphoma
Burkitt lymphoma
Splenic marginal zone lymphoma
Sezary syndrome
Burkitt lymphoma
What do CLL and myeloma have in common?
Osteolytic lesions
Light chain restriction
Cell of origin
Immunophenotype
Light chain restriction