SQs Leukocyte Disorders Flashcards
Inherited leukocyte disorders caused by a mutation in the lamin B receptor.
Pelger-Huet anomaly
Inherited leukocyte disorder that is one of a group of disorders with mutations in nonmuscle myosin heavy-chain IIA.
May-Hegglin anomaly
Inherited leukocyte disorders that might be seen in Hurler syndrome.
Alder-Reilly anomaly
A lysosomal storage disease characterized by macrophages with striated cytoplasm and storage of glucocerebroside.
Gaucher disease
The neutrophils in chronic granulomatous disease are incapable of producing:
Hydrogen peroxide
Hypochlorite
Superoxide
Individuals with X-linked SCID have a mutation that affects their ability to synthesize:
IL-2 receptor
An absolute lymphocytosis with reactive lymphocytes suggests what condition?
Viral infection
What leukocyte cytoplasmic inclusion is composed of ribosomal RNA?
Dohle bodies
The expected complete blood count results for women in active labor would include:
High total WBC count with a slight shift to the left in neutrophils
Which is true of an absolute increase in lymphocytes with reactive morphology?
a. The population of lymphocytes appears morphologically homogeneous.
b. They are usually effector B cells.
c. The reactive lymphocytes have increased cytoplasm with variable basophilia.
d. They are most commonly seen in bacterial infections.
C. The reactive lymphocytes have increased cytoplasm with variable basophilia
Leukocytosis can be caused by
a. increased movement of immature cells out of the bone marrow’s proliferative compartment
b. increased mobilization of granulocytes from the maturation-storage compartment
c. increased movement of granulocytes from the marginating pool to the circulating pool
All of the choices (hehe)
Neutrophilia can be related to a variety of conditions or disorders. Select the appropriate conditions.
a. surgery
b. burns
c. stress
All of the choices
Surgery
Burns (tissue necrosis)
Stress (physiologic)
Charcot-Leyden crystals can be found in the ff. of patients with active eosinophilic inflammation, except:
a. sputum
b. tissues
c. stool
d. AOTC
e. NOTC
None of the choices.
Monocytosis can be observed in
a. tuberculosis
b. fever of unknown origin
c. rheumatoid arthritis
d. AOTC
e. NOTC
All of the choices
Neutropenia can be observed in the ff., except:
a. bone marrow injury
b. nutritional deficiency
c. increased destruction and utilization
d. AOTC
e. NOTC
None of the choices
Indicate the abnormality based on the characteristic stated below:
Precipitated mucopolysaccharides
Alder-Reilly inclusions
Indicate the abnormality based on the characteristic stated below:
Gigantic peroxidase-positive deposits
Chediak-Higashi syndrome
Indicate the abnormality based on the characteristic stated below:
Single or multiple pale-blue staining inclusions
Dohle body inclusions
Indicate the abnormality based on the characteristic stated below:
Dohle body-like inclusions
May-Hegglin anomaly
What abnormality is being described:
Failure of the nucleus to segment/decreased nuclear segmentation
Pelger-Huet anomaly
What abnormality is being described:
Dark blue-black precipitates of RNA
Toxic granulation
What abnormality is being described:
Five or more nuclear segments
hypersegmentation
What abnormality is being described by the following condition:
Associated with frequent infections in children or young adults
Chediak-Higashi syndrome
What abnormality is being described by the following condition:
Associated with viral infections and burns
Dohle Bodies
What abnormality is being described by the following condition:
May be related to a maturational arrest in some acute infections
Pelger-Huet anomaly
What abnormality is being described by the following condition:
Associated with a deficiency of vitamin B12 or folic acid
Hypersegmentation
What is the appropriate cell type involved in this disorder:
Gaucher disease
A. Neutrophilic series
B. Monocytic-macrophagic series
Monocytic-macrophagic series
What is the appropriate cell type involved in this disorder:
Niemann-pick disease
A. Neutrophilic series
B. Monocytic-macrophagic series
Monocytic-macrophagic series
What is the appropriate cell type involved in this disorder:
Chediak-higashi syndrome
A. Neutrophilic series
B. Monocytic-macrophagic series
Neutrophilic series
What is the appropriate cell type involved in this disorder:
Chronic granulomatous disease
A. Neutrophilic series
B. Monocytic-macrophagic series
Neutrophilic series
What is the appropriate cell type involved in this disorder:
Lazy leukocyte syndrome
A. Neutrophilic series
B. Monocytic-macrophagic series
Neutrophilic series
Gaucher cells have
a. wrinkled cytoplasm
b. one to three nuclei
c. a deficiency of B-glucocerebrosidase
All of the choices :)
A peripheral blood film that shows increased neutrophils, basophils, eosinophils, and platelets is highly suggestive of __________.
Chronic Myelogenous Leukemia (CML)
What chromosome abnormality is associated with CML?
t (9;22)
A patient has a WBC count of 30x10^9/L and the following WBC differential:
Segmented neutrophils: 38%
Bands: 17%
Metamyelocytes: 7%
Myelocytes: 20%
Promyelocytes: 10%
Eosinophils: 3%
Basophils: 5%
What test would be helpful in determining whether the patient has CML?
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?
Transformation to acute leukemia
The most common mutation found in patients with primary polycythemia vera (PV) is:
JAK2 V617F
The peripheral blood in polycythemia vera (PV) typically manifests:
erythrocytosis, thrombocytosis, and granulocytosis
A patient has a platelet count of 700x10^9/L with abnormalities in the size, shape, and granularity of platelets; a WBC count of 12x10^9/L; and hemoglobin of 11g/dL. The philadelphia chromosome is not present. The most likely diagnosis is:
Essential Thrombocythemia (ET)
Complications of essential thrombocythemia include all of the following except:
a. thrombosis
b. hemorrhage
c. seizures
d. infections
d. infections
Which of the following patterns is a characteristic of the peripheral blood in patients with PMF?
a. Teardrop-shaped erythrocytes, nucleated, RBCs, immature granulocytes
b. abnormal platelets only
c. Hypochromic erythrocytes, immature granulocytes, and normal platelets
d. Spherocytes, immature granulocytes, and increased number of platelets
A. Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes
The myelofibrosis associated with PMF is a result of:
Enhanced activity of fibroblasts owing to increased stimulatory cytokines.
The most characteristic morphological features of variant lymphocytes include:
a. increased overall size, possibly 1 to 3 nucleoli, and abundant cytoplasm
b. increased overall size, round nucleus, and increased granulation in the cytoplasm
c. segmented nucleus, light-blue cytoplasm, and no nucleoli
d. enlarged nucleus, six to eight nucleoli, and dark-blue cytoplasm
a. increased overall size, possibly 1 to 3 nucleoli, and abundant cytoplasm
Match the following with the given choices:
Rieder cells
A. Niemann-pick disease and burkitt lymphoma
B. CLL
C. Leukosarcoma
D. Natural artifact
B. CLL (Chronic Lymphogenous Leukemia)
Match the following with the given choices:
Vacuolated lymphocytes
A. Niemann-pick disease and burkitt lymphoma
B. CLL
C. Leukosarcoma
D. Natural artifact
A. Niemann-pick disease and burkitt lymphoma (plus tay sachs disease and hurley syndrome)
Match the following with the given choices:
Crystalline inclusions
A. Niemann-pick disease and burkitt lymphoma
B. CLL
C. Leukosarcoma
D. Natural artifact
C. Leukosarcoma
Match the following with the given choices:
Smudge cells
A. Niemann-pick disease and burkitt lymphoma
B. CLL
C. Leukosarcoma
D. Natural artifact
D. natural artifact
Also usually seen in CLL
T cells are
A. lymphocytes
B. monocytes
C. helper or suppressor types
D. Both A and C
D. Lymphocytes & Helper or Suppressor Types
B cells are
A. lymphocytes
B. associated with antigen recognition
C. found in thymus and bone marrow
D. AOTC
All of the choices
NK cells are classified as
A. macrophages
B. monocytes
C. effector lymphocytes
D. K-type lymphocytes
C. effector lymphocytes
Which of the ff. statements is (are) true of T cells?
A. Responsible for humoral responses
B. Responsible for cellular immune responses
C. Responsible for chronic rejection in organ transplantation
D. Both A and B
E. Both B and C
E. Both B and C
Which of the ff. statements is (are) true of B cells?
A. Responsible for antibody responses
B. Protect against intracellular pathogens
C. Responsible for chronic rejection in transplantation
D. Both A and B
A. Responsible for antibody responses
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:
20%
Sudan Black B stains what component of the cell?
Lipids
MDSs (Myelodysplastic Syndromes) are common in which age group?
Older than 50 years
What is a major indication of MDS in the peripheral blood and bone marrow?
Dyspoiesis (hematopoiesis is ineffective)
An alert hematologist should recognize all of the following peripheral blood abnormalities as diagnostic clues in MDS except:
a. Oval macrocytes
b. Target cells
c. Agranular neutrophils
d. Circulating micromegakaryocytes
b. Target cells
For an erythroid precursor to be considered a ring sideroblast, the iron-laden mitochondira must encircle how much of the nucleus?
1/3
According to the WHO classification of MDS, what percentage of blasts would constitute transformation to an acute leukemia?
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?
Vitamin B12 and folate levels
Which of the following is least likely to contribute to the death of patients with MDS?
a. neutropenia
b. thrombocytopenia
c. organ failure
d. neuropathy
neuropathy
In what other hematologic disease does MDS often convert?
AML
Chronic myelomonocytic leukemia is classified in the WHO system as:
MDS/MPN
In most cases, the diagnosis of lymphoma relies on all of the ff. except:
a. Microscopic examination of affected lymph nodes
b. Immunophenotyping using immunohistochemistry or flow cytometry
c. Molecular or cytogenetic analysis
d. Peripheral blood examination and a complete blood count
d. Peripheral blood examination and a complete blood count
The most common lymphoma occurring in young adults is:
Hodgkin lymphoma
In a normal lymph node, the medulla includes predominantly:
Plasma cells
The t(11;14) is the defining feature of:
Mantle cell lymphoma
The immunophenotype of mycosis fungoides is:
An abnormal T cell immunophenotype with expression of CD4 and loss of CD7 antigen
What is the major morphologic difference between Hodgkin lymphoma and other B cell lymphomas?
The presence of numerous reactive lymphocytes and only a few malignant cells in Hodgkin lymphoma
Which morphologic diagnosis has to be confirmed with molecular studies demonstrating the presence of t(8;14)?
Burkitt lymphoma
What is the function of the germinal center?
Generation of B cells producing immunoglobulins with the highest affinity for a particular antigen through the process of somatic mutation
Marked paracortical expansion is most commonly seen in:
Dermatopathic lymphadenopathy
Monoclonal gammopathy of undetermined significance (MGUS) is best described as:
The presence of monoclonal immunoglobulin in serum with only mild bone marrow plasmacytosis
Lymphocytopenia means
a total decrease in lymphocytes
The helper subset of T lymphocytes is _________ in AIDS
a. increased
b. decreased
c. not altered
b. decreased
Match the given disorder with the following choices.
Radiation exposure
A. lymphocytosis
b. lymphocytopenia
B. lymphocytopenia
Match the given disorder with the following choices.
Infectious mononucleosis
A. lymphocytosis
b. lymphocytopenia
A. lymphocytosis
Match the given disorder with the following choices.
Cytotoxic drugs
A. lymphocytosis
b. lymphocytopenia
b. lymphocytopenia
Match the given disorder with the following choices.
Whooping cough
A. lymphocytosis
b. lymphocytopenia
A. lymphocytosis
Match the given disorder with the following choices.
Immune deficiency disorders
A. lymphocytosis
b. lymphocytopenia
b. lymphocytopenia
Match the given disorder with the following choices.
Toxoplasmosis
A. lymphocytosis
b. lymphocytopenia
a. lymphocytosis
Which of the following characterizes infectious lymphocytes?
a. an adult disorder
b. leukocytopenia in the early stages
c. lymphocyte differential counts over 95%
d. lymphoblasts on the peripheral blood smear
c. lymphocyte differential counts over 95%
AIDS is caused by
HIV-1
A definition of leukemia could include
a. an overproduction of leukocytes
b. solid, malignant tumors of the lymph nodes
c. malignant cells trespass the blood-brain barrier
d. both A and C
e. AOTC
d. both A and C
Descriptive terms for most lymphomas can include
a. a nonneoplastic proliferative disease
b. a solid malignant tumor of the lymph nodes
c. a lymphocytopenia
d. freely trespassing the blood-brain barrier
b. a solid malignant tumor of the lymph nodes
An acute leukemia can be described as being
of short duration with many immature leukocyte forms in the peripheral blood
The etiological agents of leukemias can include
a. ionizing radiation
b. certain infectious agents
c. chemical exposure to benzene
d. AOTC
d. All of the choices
HIV is associated with
AIDS
The incidence of leukemia is higher in _______.
Scandinavian versus Japanese populations
Which of the following are typical characteristics of an acute leukemia?
a. replacement of normal marrow elements by leukocytic blasts and bleeding episodes
b. blasts and immature leukocyte forms in the peripheral blood and anemia
c. leukocytosis
All of the choices :)
Match the type of leukemia with its FAB classifications
Myeloid and monocytic
M4
Match the type of leukemia with its FAB classifications
Monocytic
M5
Match the type of leukemia with its FAB classifications
Myeloid without maturation
M1
Match the type of leukemia with its FAB classifications
Lymphoblastic (one cell population)
L1
Characteristics of FAB M1 include …
Leukocytosis without maturation of the myeloid cell line in the peripheral blood
The incidence of FAB M1 is high in _______.
children younger than 18 months of age and middle-aged adults
Match the following predominant peripheral blood cell morphological appearances with its FAB classification
A mixture of myeloid and monocytic blasts
FAB M4
Match the following predominant peripheral blood cell morphological appearances with its FAB classification
Blasts of the monocytic type
FAB M5
Match the following predominant peripheral blood cell morphological appearances with its FAB classification
Many coarsely granular promyelocytes with dumbbell-shaped or bilobed nuclei
FAB M3
Match the following predominant peripheral blood cell morphological appearances with its FAB classification
Myeloblasts, promyelocytes, and myelocytes
FAB M2
Match the following predominant peripheral blood cell morphological appearances with its FAB classification
Immature leukocytic and erythrocytic cell types
FAB M6
FAB classification:
Leukemia secondary to burkitt lymphoma
FAB L3
FAB classification:
Childhood lymphoblastic leukemia
FAB L1
FAB classification:
Older children and adults
FAB L2
Chloromas are associated with
FAB M1
A common characteristic of ALL is
bone and joint pain
Chromosomal alterations with the appropriate FAB type:
t(15q+;17q)
FAB M3
Chromosomal alterations with the appropriate FAB type:
t(8q-;21q+)
FAB M2
Chromosomal alterations with the appropriate FAB type:
t(9;22)
FAB ALL
Cytochemical stain with the appropriate constituent:
Sudan black B
Lipids
Cytochemical stain with the appropriate constituent:
Myeloperoxidase
Enzymes
Cytochemical stain with the appropriate constituent:
PAS
Glycogen
The Sudan Black B cytochemical stain differentiates
acute myeloid from ALL
Myeloperoxidase differentiates
acute myelomonocytic from acute monocytic leukemia
The PAS reaction is
positive in the neutrophilic granulocytes, except blasts
Esterase (naphthol AS-D chloracetate) differentiates
granulocytic (promyelocytic to segmented neutrophils) from the monocytic line
In the nonspecific esterase staining reaction, the cells of monocytic origin are
strongly positive
Specific nature B-cell surface marker(s) membrane is
CD 79a, CD19, CD20
Patients with AML have a good prognosis is
Less than 45 years of age
Over rods are present in blast cells
Ph chromosome
The most common form of chronic leukemia in Western countries is
lymphocytic
CLL is classically a
null cell disorder
CLL symptoms frequently include
absolute lymphocytosis, malaise, and low-grade fever
Characteristics of malignant lymphoma typically include
Overproliferation of lymphoctes and lymph node involvement
A lymphoma characterized by Reed-Sternberg cells and occurs more frequently in males than in females
Hodgkin disease
Rare forms of lymphoma
Non-hodgkin lymphoma and mycosis fungoides
A disorder of plasma cells
multiple myeloma
The abnormal protein frequently found in the urine of persons with multiple myeloma is
Bence jones
Waldenstrom macroglobulinemia is characterized by increased levels of
IgM
What cluster designations are positive in typical HCL?
CD25, CD22, CD19, CD20
Myeloproliferative neoplasms are characterized by all of the following except
a. clonal disorders
b. they may evolve into acute leukemia
c. initial increase of immature cells
d. increased production of mature cells
c. initial increase of immature cells
In CML, the total leukocyte count is
extremely increased
Primary myelofibrosis differs from other types of MPN in which of the following ways?
Marrow fibrosis is greatly increased
A remarkable characteristic of PV compared with other types of MPNs
Extremely increased erythrocyte mass
Predominant feature of essential thrombocythemia compared with other types of MPNs
extremely increased number of platelets
In MPN, the test results of disorders of hemostasis and coagulation that are most likely to be abnormal are:
Increased APTT, decreased factor V level, and increased concentration of antithrombin III in many
Interferon alfa has been shown to
suppress proliferation of hematopoietic progenitor cells
A leukemia of long duration that affects the neutrophilic granulocytes is referred to as
CML
The alkaline phosphatase cytochemical staining reaction is used to differentiate between
CML and severe bacterial infections
Patients with the initial phase of CML are prone to
low-grade fevers, night sweats, and splenic infarction
The total leukocyte count in CML usually is ______x10^9/L
> 50
The philadelphia chromosome is typically associated with
CML
Patients with PV suffer from
hypervolemia
Hyperviscosity can produce
dizziness
The major criteria for diagnosis of PV include all of the following except
a. increased red blood cell mass
b. presence of JAK2V617F
c. hypercellular bone marrow
d. splenomegaly
d. splenomegaly
Increased blood viscosity in patients with PV can cause a dangerous condition of
vascular occlusion
The level of erythropoietin in the urine is ________ in patients with PV compared with other kinds of polycythemia
decreased
Patients with PV demonstrate an _________ of hemosiderin in the bone marrow
absence
Life expectancy after diagnosis of treated patients with PV
More than 10 years
Primary treatment for PV
therapeutic phlebotomy
Primary myelofibrosis is also called
agnogenic myeloid metaplasia
Incidence of primary myelofibrosis is known to increased after exposure to
benzene
Predominant clinical manifestation of primary myelofibrosis
anemia, splenomegaly, medullary fibrosis
Most constant feature of primary myelofibrosis
dysmegakaryocytopoiesis
Least common form of MPN
essential thrombocythemia
Major criterion for the diagnosis of essential thrombocythemia
persistent increase of platelets in the peripheral blood
Most common disorder in patients with essential thrombocythemia
neurological manifestations
The bone marrow architecture in essential thrombocythemia is similar to the architecture seen in
CML
