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hematopoietic diseases Flashcards

1
Q

Average volume per cell, expressed in femtoliters.

A

Mean cell volume (MCV)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423

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2
Q

A reduction in the oxygen-transporting capacity of blood.

A

Anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 422

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3
Q

The average content of hemoglobin per red cell, expressed in picograms.

A

Mean cell hemoglobin (MCH)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423

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4
Q

The average concentration of hemoglobin in a given volume of packed red cells, expressed in g/dL.

A

Mean cell hemoglobin concentration (MCHC)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423

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5
Q

The coefficient of variation of red cell volume.

A

Red cell distribution width (RDW)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423

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6
Q

Anemia of acute blood loss is described as ______.

A

Normocytic, normochromic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423

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7
Q

Life span of a normal red cell.

A

120 days(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424

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8
Q

Anemia characterized by an increased rate of cell destruction, a compensatory increase in erythropoeisis that results in reticulocytosis, and retention of products of cell destruction, including iron.

A

Hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424

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9
Q

A circulating protein that binds and clears free hemoglobin.

A

Haptoglobin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424

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10
Q

Hemolysis that can result from mechanical trauma, or biochemical or physical agents that damage the red cell membrane.

A

Intravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424

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11
Q

Hemolysis which takes place largely within phagocytic cells of the spleen and liver.

A

Extravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424

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12
Q

This disorder is characterized by an intrinsic defect in the red cell membrane, that renders the cells spheroidal, less defomable and vulnerable to splenic sequestration and destruction.

A

Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424

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13
Q

Small, dark nuclear remnants seen within red cells in PBS of hereditary spherocytosis.

A

Howell-Jolly bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425

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14
Q

On PBS, red cells are spherical which lack central pallor, and they show increased osmotic fragility when placed in hypotonic salt solutions.

A

Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425

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15
Q

Structural proteins that are defective in hereditary spherocytosis.

A

Spectrin and ankyrin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425

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16
Q

This results from substitution of valine for glutamic acid at the 6th position of the B-chain, producing HbS.

A

Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 426

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17
Q

Bizarre, elongated, spindled or boat-shaped cells on PBS.

A

Sickel cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427

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18
Q

Prominent cheekbones and changes in skull resembling a “crew-cut” skull x-ray.

A

Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427

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19
Q

Patients with sickle cell disease are predisposed to infections caused by these type of bacteria.

A

Encapsulated bacteria(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428

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20
Q

Treatment for sickle cell disease by increasing levels of HbF.

A

Hydroxyurea(TOPNOTCH)

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21
Q

Treatment for sickle cell disease by increasing levels of HbF.

A

Hydroxyurea(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428

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22
Q

Feared complication of sickle cell disease which can be trigerred by pulmonary infections or fat emboli from necrotic marrow that secondarily involve the lung.

A

Acute chest syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428

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23
Q

Major complication of sickle cell disease which occurs in the setting of acute chest syndrome, causing ischemic injury to the CNS.

A

CNS stroke(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428

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24
Q

Represents a sudden but usually temporary cessation of erythropoeisis, usually trigerred by parvovirus B19 infections in patients with sickle cell disease.

A

Aplastic crises(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428

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25
Q

In beta thalassemia, an individual who inherits one abnormal allele (out of 2) has this asymptomatic to mildly symptomatic condition.

A

B- Thalassemia minor/trait(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429

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26
Q

Individuals with B-thalassemia who inherit two abnormal alleles, with severe anemia requiring regular blood tranfusions.

A

B- Thalassemia major(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429

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27
Q

Red cells with a central, dark-red puddle due to collection of hemoglobin.

A

Target cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430

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28
Q

Target cells are often seen in this condition.

A

B-thalassemia minor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430

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29
Q

In the PBS of this condition, nucleated red cells (normoblasts) are seen, which reflect underlying erythropoeisis.

A

B-thalassemia major(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430

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30
Q

Anemia of beta thalassemia.

A

Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430

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31
Q

Disease caused by deletion of 3 alpha globin genes.

A

Hemoglobin H disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431

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32
Q

Condition caused by deletion of 1 alpha globin gene.

A

Silent carrier(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431

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33
Q

Condition caused by deletion of 2 alpha globin genes.

A

Alpha thalassemia trait(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431

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34
Q

Condition caused by deletion of all four alpha globin genes.

A

Hydrops fetalis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429

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35
Q

Precipitates of denatured globin seen in RBC’s.

A

Heinz bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432

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36
Q

Heinz bodies are seen in the blood smear of this condition.

A

G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432

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37
Q

Bite cells are seen in ________.

A

G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432

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38
Q

A rare disorder of unknown etiology, causes hemolytic anemia which results from an acquired membrane defect secondary to a mutation that affects myeloid stem cells. Hemolysis occurs during sleep.

A

Paroxysmal nocturnal hemoglobinuria(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432

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39
Q

Hemolysis caused by IgG or IgA antiodies that are active at 37 degC, which results in opsonization of red cells by the autoantibodies.

A

Warm antibody immunohemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433

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40
Q

Anemia caused by low-affinity IgM which bind to red cell membranes only at temp <30degC, commonly experienced by distal parts of the body.

A

Cold antibody immunohemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433

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41
Q

Anemia observed in a variety of pathologic states, in which small vessels become particularly obstructed.(e.g. DIC, malignant HTN, SLE, etc.)

A

Microangiopathic hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433

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42
Q

Schistiocytes, burr cells, helmet cells, triangle cells are seen in this condition.

A

Microangiopathic hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433

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43
Q

X-linked disorder in which red cells are unusually susceptible to damage cause by oxidants.

A

G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432

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44
Q

Red blood cells in iron deficiency anemia.

A

Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 436

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45
Q

Diagnostic criteria for iron deficiency anemia:____ Ferritin____Serum iron level____Transferrin saturation____Total Iron Binding Capacity (TIBC)

A

Low ferritinLow serum iron levelsLow transferrin saturationIncreased TIBC(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 435

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46
Q

Diagnostic criteria for anemia of chronic disease:____ Ferritin____Serum iron level____Transferrin saturation____Total Iron Binding Capacity (TIBC)

A

Increased ferritinLow serum iron levelsLow transferrin saturationDecreased TIBCNormocytic, normochromic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 435

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47
Q

Principal causes of megaloblastic anemia.

A

Folate deficiencyVitamin B12 deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437

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48
Q

Bone marrow is markedly hypercellular as a sult of increased number of megaloblasts, which are large cells that have delicate, finely reticulated nuclear chromatin and abundant basophilic cytoplasm.

A

Megaloblastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437

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49
Q

PBS finding in neutrophils and red cells of patients with megaloblastic anemia.

A

Hypersegmented neutrophils, large, egg-shaped macro-ovalocytes(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437

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50
Q

Difference between megloblastic and pernicious anemia.

A

Presence of neurologic abnormalities in pernicious anemia.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438

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51
Q

Deficiency in folate causes this type of anemia.

A

Megaloblastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438

52
Q

Deficiency in Vitamin B12 causes this type of anemia.

A

Pernicious anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438

53
Q

Etiology of pernicious anemia.

A

1.Vitamin B12 malabsorption secondary to autoantibodies against parietal cells and intrinsic factor which is needed in its absorption.2. Gastrectomy or ileal resection(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438

54
Q

Principal neurologic lesion in pernicious anemia.

A

Demyelination of posterior and lateral columns of the spinal cord.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439

55
Q

Bone marrow is markedly hypocellular, with >90% of the intertrabecular space being occupied by fat.

A

Aplastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439

56
Q

Tear drop cells are also called __________.

A

Dacrocytes(TOPNOTCH)

57
Q

Tear drop cells are also called __________.

A

Dacrocytes(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440

58
Q

Dacrocytes are found in peripheral blood of patients with this type of anemia.

A

Myelophthisic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440

59
Q

Increase in blood concentration of red cells, with an increase in Hgb concentration.

A

Polycythemia or erythrocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441

60
Q

Polycythemia secondary to reduced plasma volume.

A

Relative polycythemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441

61
Q

Polycythemia secondary to abnormal proliferation of myeloid stem cells and low erythropoeitin levels.

A

Primary polycythemia (Absolute)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441

62
Q

Polycythemia secondary to increased erythropoeitin levels due to lung disease, high-altitude living, cyanotic heart disease and EPO secreting tumors.

A

Secondary polycythemia (Absolute)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441

63
Q

Total white cell count is reduced to 1000 cells/uL. Affected persons are extremely susceptible to bacterial and fungal infections.

A

Neutropenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441

64
Q

A self-limited disease of adolescents and young adults that is causd by B lymphocytotropic EBV characterized by fever, sore throat and generalized lymphadenitis, an increase of atypical lymphocytes in blood and an antibody and T cell response to EBV.

A

Infectious mononucleosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 442

65
Q

Cells with abundant cytoplasm 12-16um in diameter that contains azurophilic granules, and an oval, indented or folded nucleus.

A

Atypical lymphocytes (Infectious mononucleosis)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 442

66
Q

Inflamed nodes are swollen, gray-red and engorged. There are large germinal centers containing numerous mitotic figures. Affected nodes are tender and fluctuant if with extensive abscess formation.

A

Acute nonspecific lymphadenitis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444

67
Q

This pattern is associated with infections or inflammatory processes that activate B cells which create a follicular or germinal center reaction. Lymph node architecture is preserved, lymphoid nodules vary in shape and size, mixed lymphocytic infiltrates with prominent phagocytic and mitotic activity within germinal centers.

A

Follicular hyperplasia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444

68
Q

Reactive pattern characterized by distention and prominence of the lymphatic sinusoids, due to marked hypertrophy of lining endothelial cells and an infiltrate of histiocytes. Encountered in lymph nodes draining cancers.

A

Sinus histiocytosis(TOPNOTCH)

69
Q

Characterized by reactive changes within T-cell regions of the lymph node, usually encountered during viral infections, following certain vaccinations, and immune reactions induced by certain drugs.

A

Paracortical hyperplasia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444

70
Q

Formation of sarcoid-like ganulomas that undergo central necrosis associated with neutrophil accumulation. This irregular stellate necrotizing granuloma is seen in patients presenting with a raised inflammatory node, vesicle or eschar at site of injury. History of exposure to cats.

A

Cat scratch disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444

71
Q

Causative agent for cat scratch disease(TOPNOTCH)

A

Bartonella henselae(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444

72
Q

Lymphoblasts with irregular nuclear contours, condensed chromatin , small nucleoli and scant agranular cytoplasm. Blasts compose >25% of marrow cellularity. Most common childhood leukemia.

A

Acute lymphocytic leukemia (ALL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 447

73
Q

Frequent small “cleaved” cells mixed with large cells, growth pattern nodular, centroblasts present. Occurs in older adults, usually involves nodes, marrow, spleen. Associated with t(14;18).

A

Follicular lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 451

74
Q

Small to intermediate-sized irregular lymphocytes growing in a diffuse pattern, no centroblasts and proliferation centers. Occurs mainly in older males, GI tract commonly affected. Associated with t(11;14).

A

Mantle cell lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 452

75
Q

Plasma cells in sheets, with prominent nucleoli or inclusion containing Ig. Presents as disseminated bone disease, with destructive lytic lesions.

A

Plasmacytoma / plasma cell myeloma(TOPNOTCH)

76
Q

Intermediate-sized round lymphoid cells with 2-5 prominent nucleoli. Nuclear remnants phagocytosed by interspersed macrophages with abundant clear cytoplasm, “starry sky pattern”

A

Burkitt lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 453

77
Q

Sheets of small, round lymphocytes and scattered ill-defined foci of larger, actively dividing cells diffusely efface involved LN. A foci of mitotically active cells called proliferation centers are pathognomonic.

A

Small lymphocytic leukemia (SLL) / Chronic lymphocytic leukemia (CLL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450

78
Q

Fragile neoplastic lymphocytes that are frequently disrupted during smear preparation.

A

Smudge cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450

79
Q

Smudge cells are seen in this type of leukemia.

A

CLL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450

80
Q

Tumor cells have large nuclei with open chromatin and prominent nucleoli. Most important type of lymphoma in adults, accounting to ~50% of adult NHL.

A

Diffuse large B-cell lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 452

81
Q

Multifocal destructive bone lesions seen as punched-out defects in bone radiographs.

A

Multiple myeloma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 455

82
Q

Excess lightvor heavy chains along with complete Igs synthesized by neoplastic plasma cells.

A

Bence-Jones proteins(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 454

83
Q

Cells with two mirror-image nuclei or nuclear lobes, each containing a large acidophilic nucleolus surrounded by a distinctive clear zone, imparting a n owl-like appearance.

A

Reed-Sternberg cell(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 456

84
Q

A distinctive groups of neoplasms that arise almost invariably in a single lymph node or chain of lymph nodes and spread characteristically in a stepwise fashion to anatomically contiguous nodes.

A

Hodgkin Lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 456

85
Q

Most common form of Hodgkin lymphoma, characterized by a large cell which has a single multilobate nucleus with small nucleoli and an abundant pale-staining cytoplasm called lacunar cells.

A

Nodular sclerosis HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 457

86
Q

Most common form of HL in patients greater than 50 years old, with male predominance, plentiful RS cells and heterogenous cellular infiltrates.

A

Mixed cellularity HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 458

87
Q

Cells found in adult T-cell lymphoma which appear to have multilobulated nuclei.

A

Cloverleaf or flower cell(TOPNOTCH)

88
Q

Subgroup of HL characterized by a large number of small resting lymphocytes admixed with a variable number of benign histiocytes. Variant RS cells described as multilobed, puffy nucleus Which appears like a “popcorn”. Excellent prognosis.

A

Lymphocyte-predominance HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 458

89
Q

Cells with fiery red cytoplasm.

A

Flame cells(TOPNOTCH)

90
Q

Cells with pink globular cytoplasmic inclusions.

A

Russell bodies(TOPNOTCH)

91
Q

Cells with blue globular nuclear inclusions.

A

Dutcher bodies(TOPNOTCH)

92
Q

Multiple nuclei, prominent nucleoli, and cytoplasmic droplets containing Ig.

A

Bizarre, multinucleated cells(TOPNOTCH)

93
Q

Bizarre multinucleated cells, flame cells, Russel bodies and Dutcher bodies are all seen in what disease?

A

Multiple myeloma(TOPNOTCH)

94
Q

Bone marrow aspirate shows hypercellular martow packed with myeloblasts and azurophilic needle-like material called Auer rods.

A

Acute Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 462

95
Q

Leukemia of children most responsive to chemotherapy.

A

Acute Lymphoblastic Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 461

96
Q

Mature B-cell tumor in the elderly, where cells have fine hair-like projections (hairy cells).

A

Hairy cell leukemia(TOPNOTCH)

97
Q

Hodgkin lymphoma subgroup most commonly associated with EBV infection.

A

Lymphocyte depleted(TOPNOTCH)

98
Q

Mature B-cell tumor in the elderly, where cells have fine hair-like projections (hairy cells).

A

Hairy cell leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 459

99
Q

t(9;22) is also called ______.

A

Philadelphia chromosome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 465

100
Q

Hodgkin lymphoma subgroup with highest count of RS cells.

A

Mixed cellularity type(TOPNOTCH)

101
Q

This correlates with good prognosis in Hodgkin lymphoma.

A

High Lymphocyte : Reed-Sternberg cell ratio(TOPNOTCH)

102
Q

Hodgin lymphoma subgroup not associated with EBV.

A

Nodular sclerosis and lymphocyte predominant(TOPNOTCH)

103
Q

Hodgkin lymphoma subgroup with poorest prognosis.

A

Lymphocyte depleted HL(TOPNOTCH)

104
Q

Tumor of the thymus associated with myastheni gravis and pure red cell aplasia.

A

Thymoma(TOPNOTCH)

105
Q

Pentalaminar tubules, often with a dilated terminal end (tennis racket-like appearance).

A

Birbeck granules(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467

106
Q

Proliferative disorder of the dendritic cells which has birbeck granules.

A

Langerhans Cell Histiocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467

107
Q

Pathology behind polycythemia vera.

A

Mutation in tyrosine kinase JAK2, which acts in signalling pathways of the erythropoeitin receptors, rendering them hypersensitive to erythropoeitin.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 466

108
Q

Treatment for polycythemia vera.

A

Phlebotomy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 466

109
Q

Collection of aggressive tumors that are comprised of immature myeloblasts which replace the marrow and suppress normal hematopoiesis.

A

Acute Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467

110
Q

Myeloid tumor arising from a pluripotent stem cell associated with mutatios of the BCR-ABL gene. If untreated, may progress to a blast crisis.

A

Chronic Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467

111
Q

Most common myelodysplastic syndrome. A myeloid tumor in which abnormal megakaryocytes stimulate marrow fibroblasts to release collagen, replacing the marrow space, leading to pancytopenia and extramedullary hematopoeisis.

A

Myeloid metaplasia with Myelofibrosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467

112
Q

Other name for acute disseminated Langerhans cell histiocytosis.

A

Letterer-Siwe disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 468

113
Q

Caused by a systemic activation of coagulation pathways, leading to formation of thrombi throughout the microcirculation.

A

Disseminated intravascular coagulation(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 469

114
Q

Characterized by spontaneous bleeding, prolonged bleeding time, and normal PT and PTT.

A

Thrombocytopenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 471

115
Q

Drug-induced disorder caused by IgG antibodies that bind to platelet factor IV on platelet surfaces, which activates platelets and induce their aggregation.

A

Heparin-Induced Thrombocytopenia (HIT)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472

116
Q

Associated with pentad of fever, thrombocytopenia, microangipathic hemolytic anemia, transient neurologic deficits and renal failure.

A

Thrombotic thrombocytic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472

117
Q

Associated with childhood onset microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. No neurologic symptoms.

A

Hemolytic uremic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472

118
Q

Caused by antiplatelet antibodies directed against glycoproteins IIb-IIIa, Ib-IX.

A

Immune thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472

119
Q

Decreased gp Ib leads to defective platelet adhesion, associated with decreased platelet count.

A

Bernard-Soulier Syndrome(TOPNOTCH)

120
Q

Caused by deficiency of ADAMTS13, a vWF metalloprotease.

A

Thrombotic thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472

121
Q

Caused by shiga-like toxin in EHEC (E. coli O157:H7) from improperly cooked burgers.

A

Hemolytic uremic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472

122
Q

Decreased gp IIb-IIIa leads to defective platelet aggregation, associated with normal platelet count.

A

Glanzmann thrombasthenia(TOPNOTCH)

123
Q

Most common bleeding disorder.

A

vWF disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474

124
Q

Most common hereditary disease associated with life threatening bleeding,

A

Hemophilia A(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474

125
Q

An X-linked recessive disorder caused by reduction in factor VII activity.

A

Classic Hemophilia/ Hemophilia A(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474

126
Q

An X-linked disorder caused by deficiency of Factor IX, or Christmas factor. Bleeding time is normal, PTT is prolonged.

A

Hemophilia B/ Christmas Disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474

127
Q

A state associated with excessive removal of formed elements of blood, resulting in anemia, leukopenia or thrombocytopenia.

A

Hypersplenism(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 476

patho (23 decks)

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