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Topnotch Pathology > The Hematopoietic and Lymphoid Systems > Flashcards

The Hematopoietic and Lymphoid Systems 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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19
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 ferritin
Low serum iron levels
Low transferrin saturation
Increased 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 ferritin
Low serum iron levels
Low transferrin saturation
Decreased TIBC
Normocytic, normochromic anemia

(TOPNOTCH)

Robbins Basic Pathology, 8th Ed. p. 435

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47
Q

Principal causes of megaloblastic anemia.

A

Folate deficiency
Vitamin 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

55
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

67
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

68
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

74
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

75
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

86
Q

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

A

Cloverleaf or flower cell

TOPNOTCH

87
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

88
Q

Cells with fiery red cytoplasm.

A

Flame cells

TOPNOTCH

89
Q

Cells with pink globular cytoplasmic inclusions.

A

Russell bodies

TOPNOTCH

90
Q

Cells with blue globular nuclear inclusions.

A

Dutcher bodies

TOPNOTCH

91
Q

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

A

Bizarre, multinucleated cells

TOPNOTCH

92
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

95
Q

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

A

Hairy cell leukemia

TOPNOTCH

96
Q

Hodgkin lymphoma subgroup most commonly associated with EBV infection.

A

Lymphocyte depleted

TOPNOTCH

97
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

98
Q

t(9;22) is also called ______.

A

Philadelphia chromosome

(TOPNOTCH)

Robbins Basic Pathology, 8th Ed. p. 465

99
Q

Hodgkin lymphoma subgroup with highest count of RS cells.

A

Mixed cellularity type

TOPNOTCH

100
Q

This correlates with good prognosis in Hodgkin lymphoma.

A

High Lymphocyte : Reed-Sternberg cell ratio

TOPNOTCH

101
Q

Hodgin lymphoma subgroup not associated with EBV.

A

Nodular sclerosis and lymphocyte predominant

TOPNOTCH

102
Q

Hodgkin lymphoma subgroup with poorest prognosis.

A

Lymphocyte depleted HL

TOPNOTCH

103
Q

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

A

Thymoma

TOPNOTCH

104
Q

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

A

Birbeck granules

(TOPNOTCH)

Robbins Basic Pathology, 8th Ed. p. 467

105
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

118
Q

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

A

Bernard-Soulier Syndrome

TOPNOTCH

119
Q

Caused by deficiency of ADAMTS13, a vWF metalloprotease.

A

Thrombotic thrombocytopenic purpura

(TOPNOTCH)

Robbins Basic Pathology, 8th Ed. p. 472

120
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

121
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

Topnotch Pathology (25 decks)

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