XII - The Hematopoietic and Lymphoid Systems Flashcards
1
Q
True or False. Age younger 2 years is associated with a worse prognosis in ALL.
A
True(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
2
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
3
Q
Hodgkin lymphoma subgroup with poorest prognosis.
A
Lymphocyte depleted HL(TOPNOTCH)
4
Q
In primary myelofibrosis, marrow fibroblasts are stimulated to proliferate by PDGF and TGF-beta released from (A) granulocyte precursors (B) erythroid precursors (C) lymphoid cells (D) neoplastic megakaryocytes
A
neoplastic megakaryocytes (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 466
5
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
6
Q
Lymphoblasts with irregular nuclear contours, condensed chromatin , small nucleoli and scant agranular cytoplasm. SEE SLIDE 12.10. Blasts compose >25% of marrow cellularity. Most common childhood leukemia.
A
Acute lymphocytic leukemia (ALL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 447
7
Q
t(9;22) is also called ______.
A
Philadelphia chromosome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 465
8
Q
LYMPHOBLAST or MYELOBLAST: More cytoplasm, which often contains granules
A
Myeloblast (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 433
9
Q
Type of lymph node hyperplasia characterized by distention of sinusoids due to macrophage infiltration and hypertrophy of endothelial cells. Encountered in cancer.
A
Sinus histiocytosis (TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 428
10
Q
Anemia caused by low-affinity IgM which bind to red cell membranes only at temp
A
Cold antibody immunohemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
11
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
12
Q
Single most important factor in the pathogenesis of renal failure in Multiple myeloma
A
Bence-Jones proteinuria(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 600
13
Q
A 52 y/o female presents with dragging sensation in the abdomen associated with anemia, weakness, and weight loss. Chromosomal analysis showed presence of BCR-ABL fusion gene. What is the most likely diagnosis?
A
CML (TOPNOTCH)
14
Q
Most common nutritional disorder in the world
A
Iron Deficiency Anemia(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 649
15
Q
The most common trigger for episodic hemolysis in G6PD Deficiency
A
Infection(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 634
16
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)
17
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
18
Q
Plasma cell neoplasm commonly associated with lytic bone lesions, pathologic fractures, chronic pain, hypercalcemia, renal failure, and acquired immune abnormalities.
A
Multiple Myeloma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 599
19
Q
What laboratory finding differentiate leukemoid reaction from CML?
A
Elevated leukocyte alkaline phosphatase. (TOPNOTCH)
20
Q
Bizarre, elongated, spindled or boat-shaped cells on PBS. SEE SLIDE 12.3
A
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427
21
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
22
Q
An 18 y/o male presents with easy fatigability, fever, and cutaneous bleeding. Bone marrow biopsy showed 40% myeloblasts. What is the most likely diagnosis?
A
AML (TOPNOTCH)
23
Q
Most common presenting symptoms of this condition are spontaneous bleeding from mucous membranes, excessive bleeding from wounds, or menorrhagia. Bleeding tendency often goes unnoticed until some hemostatic stress, such as surgery, reveals its presence. Patients may have defects in platelet function despite a normal platelet count, prolonged PTT.
A
von Willebrand disease (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 662
24
Q
Most common leukemia of adults which usually presents with bone marrow and lymph node involvement.
A
Small lymphocytic lymphoma/CLL(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
25
Anemia of acute blood loss is described as ______.
Normocytic, normochromic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
26
Disease that presents as multifocal destructive bone lesions seen as punched-out defects on imaging. Renal involvement is also prominent, causing production of proteinaceous casts in the DCT and collecting ducts.
Multiple myeloma(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 438
27
Bone marrow aspirate shows hypercellular martow packed with myeloblasts and azurophilic needle-like material called Auer rods. SEE SLIDE 12.21
Acute Myelogenous Leukemia. Particularly numerous in acute promyelocytic leukemia. (TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 462
28
A 5 y/o male presented with malaise and low grade fever for 10 days. He was diagnosed with toxoplasmosis and was given pyrimethamine and sulfadiazine. Three days after, patient presented with jaundice and dark urine. PBS showed Heinz bodies and bite cells. What is the most likely diagnosis?
G6PD Deficiency(TOPNOTCH)
29
A reduction in the oxygen-transporting capacity of blood.
Anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 422
30
Increase in blood concentration of red cells, with an increase in Hgb concentration.
Polycythemia or erythrocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
31
Patient presents with anemia, splenomegaly, and jaundice. Peripheral blood smear shows small, dark-staining red cells lacking the central zone of pallor. Father had prolonged jaundice since childhood. What is the most likely diagnosis?
Hereditary spherocytosis(TOPNOTCH)
32
Bizarre multinucleated cells, flame cells, Russel bodies and Dutcher bodies are all seen in what disease? SEE SLIDES 12.19 and 12.20
Multiple myeloma(TOPNOTCH)
33
Target cells are often seen in this condition.
B-thalassemia minor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
34
Most common etiology of aplastic anemia
Idiopathic (TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 424
35
Difference between megaloblastic and pernicious anemia.
Presence of neurologic abnormalities in pernicious anemia.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
36
The coefficient of variation of red cell volume.
Red cell distribution width (RDW)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
37
LYMPHOBLAST or MYELOBLAST: Often periodic acid-Schiff (PAS) positive
Lymphoblast (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 433
38
An X-linked disorder caused by deficiency of Factor IX, or Christmas factor. Bleeding time is normal, PTT is prolonged.
Hemophilia B/ Christmas Disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
39
Treatment for sickle cell disease by increasing levels of HbF.
Hydroxyurea(TOPNOTCH)
40
Patients with sickle cell disease are predisposed to infections caused by these type of bacteria.
Encapsulated bacteria(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
41
Tumor cells have large nuclei with open chromatin and prominent nucleoli. Most important type of lymphoma in adults, accounting to ~50% of adult NHL.
Diffuse large B-cell lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 452
42
Intermediate-sized round lymphoid cells with 2-5 prominent nucleoli. High rates of proliferation and apoptosis are characteristic. Nuclear remnants phagocytosed by interspersed macrophages with abundant clear cytoplasm, "starry sky pattern" SEE SLIDE 12.13.
Burkitt lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 453
43
Two major mechanisms that trigger DIC
Release of tissue factor and widespread endothelial injury(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 663
44
Bone marrow is markedly hypocellular, with >90% of the intertrabecular space being occupied by fat. SEE SLIDE 12.8
Aplastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439
45
Very aggressive tumor of mature B cells that usually arise at extranodal sites; strongly associated with translocations involving MYC proto-oncogene
Burkitt Lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
46
Diagnostic criteria for anemia of chronic disease:____ Ferritin____Serum iron level____Transferrin saturation____Total Iron Binding Capacity (TIBC)
Increased ferritinLow serum iron levelsLow transferrin saturationDecreased TIBCNormocytic, normochromic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 435
47
Hemolysis and vaso-occlusive crisis are common in this form of anemia caused by mutaion of glutamic acid to valine.
Sickle-cell anemia(TOPNOTCH)
48
Caused by shiga-like toxin in EHEC (E. coli O157:H7) from improperly cooked burgers.
Hemolytic uremic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
49
Causative agent for cat scratch disease(TOPNOTCH)
Bartonella henselae(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
50
Most feared complication of thrombocytopenia
Intracranial bleeding (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 657
51
Associated with 15:17 translocation. 80% of patients are cured with all-trans retinoic acid and arsenic trioxide treatment.
Acute promyelocytic leukemia (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 444
52
HODGKIN or NON-HODGKIN LYMPHOMA: Extranodal involvement common
NON-HODGKIN (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 442
53
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. SEE SLIDE 12.14.
Small lymphocytic leukemia (SLL) / Chronic lymphocytic leukemia (CLL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
54
Presents signs and symptoms of anemia, thrombocytopenia, and neutropenia. Splenomegaly is characteristically absent. And the red cell are usually macrocytic and normochromic. (+) Reticulocytopenia. Bone marrow is hypocellular. What is the condition described?
Aplastic anemia(TOPNOTCH)Robbins Basic Pathology, 9th ed. p. 654
55
Antibody that can be detected in EBV infection, which is used as a diagnostic test
Heterophil antibody (TOPNOTCH) Robbins Basic Pathology, 9th ed 426
56
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.
Sinus histiocytosis(TOPNOTCH)
57
Peripheral smear reveals red cells with bizarre shapes (poikilocytes, teardrop cells), immature erythroid and white cell precursors (leukoerythroblastosis). In advanced cases, bone marrow is hypocellular and fibrotic.
Primary myelofibrosis (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 446
58
Mature B-cell tumor in the elderly, where cells have fine hair-like projections (hairy cells). SEE SLIDE 12.22
Hairy cell leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 459
59
A state associated with excessive removal of formed elements of blood, resulting in anemia, leukopenia or thrombocytopenia.
Hypersplenism(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 476
60
Leading cause of disease-related death in individuals with PNH
Thrombosis(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 642
61
HODGKIN or NON-HODGKIN LYMPHOMA: Often localized to a single axial group of nodes
HODGKIN (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 442
62
Abrupt stormy onset, symptoms of depressed marrow function, and mass effects by neoplastic infiltration , including bone pain are more common in ALL or AML?
ALL (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
63
Most common type of cancer in children; highly aggressive tumors manifest with signs and symptoms of bone marrow failure, or as rapidly growing masses
Acute lymphoblastic leukemia/Lymphoblastic lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
64
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. SEE SLIDE 12.18.
Lymphocyte-predominance HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 458
65
LYMPHOBLAST or MYELOBLAST: Coarse and clumped chromatin with scant cytoplasm
Lymphoblast (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 433
66
Cells with pink globular cytoplasmic inclusions, seen in Multiple myeloma.
Russell bodies. SEE SLIDE 12.20 (TOPNOTCH)
67
Most common leukemia of adults which usually presents with bone marrow and lymph node involvement.
Follicular lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
68
Intracellular inclusions that are made of oxidized hemoglobin proteins that precipitated, seen in G6PD deficiency
Heinz bodies. SEE SLIDE 12.4. (TOPNOTCH) Robbins Basic Pathology, 9th ed., p 416
69
Bone marrow is markedly hypercellular as a result of increased number of megaloblasts, which are large cells that have delicate, finely reticulated nuclear chromatin and abundant basophilic cytoplasm.
Megaloblastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
70
Condition caused by deletion of all four alpha globin genes.
Hydrops fetalis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
71
Feared complication of sickle cell disease which can be trigerred by pulmonary infections or fat emboli from necrotic marrow that secondarily involve the lung.
Acute chest syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
72
Characterized by spontaneous bleeding, prolonged bleeding time, and normal PT and PTT.
Thrombocytopenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 471
73
Principal neurologic lesion in pernicious anemia.
Demyelination of posterior and lateral columns of the spinal cord.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439
74
Dacrocytes are found in peripheral blood of patients with this type of anemia.
Myelophthisic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440
75
Schistiocytes, burr cells, helmet cells, triangle cells are seen in this condition. SEE SLIDE 12.6
Microangiopathic hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
76
Polycythemia secondary to increased erythropoeitin levels due to lung disease, high-altitude living, cyanotic heart disease and EPO secreting tumors.
Secondary polycythemia (Absolute)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
77
Total white cell count is reduced to 1000 cells/uL. Affected persons are extremely susceptible to bacterial and fungal infections.
Neutropenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
78
Type of lymph node hyperplasia characterized by immune reactions involving the T cell regions. Commonly caused encountered in viral infections, vaccinations, and drug-induced immune reactions.
Paracortical hyperplasia(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 428
79
Cells with fiery red cytoplasm, seen in Multiple myeloma. SEE SLIDE 12.19
Flame cells(TOPNOTCH)
80
Multiple nuclei, prominent nucleoli, and cytoplasmic droplets containing Ig.
Bizarre, multinucleated cells(TOPNOTCH)
81
Life span of a normal red cell.
120 days(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
82
Proliferative disorder of the dendritic cells which has birbeck granules. SEE SLIDE 12.23
Langerhans Cell Histiocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
83
Caused by a systemic activation of coagulation pathways, leading to formation of thrombi throughout the microcirculation.
Disseminated intravascular coagulation(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 469
84
Anemia characterized by an increased rate of cell destruction. There is a compensatory increase in erythropoeisis (seen as inceased reticulocyte count), and retention of cell destruction products, like iron.
Hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
85
High levels of this protein is seen in anemia of chronic disease. It is responsible for blocking the transfer of iron to erythroid precursors, hence causing anemia.
Hepcidin (TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 421
86
Pentalaminar tubules, often with a dilated terminal end (tennis racket-like appearance).
Birbeck granules. SEE SLIDE 12.23(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
87
Most common hereditary disease associated with life threatening bleeding,
Hemophilia A(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
88
Heinz bodies are seen in the blood smear of this condition.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
89
Average volume per red blood cell
Mean cell volume (MCV)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
90
Deficiency in folate causes this type of anemia.
Megaloblastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
91
Small to intermediate-sized irregular lymphocytes growing in a diffuse pattern, no centroblasts and proliferation centers. SEE SLIDE 12.12. Occurs mainly in older males, GI tract commonly affected. Associated with t(11;14) that results in overexpression of cyclin D1, a regulator of the cell cycle.
Mantle cell lymphoma(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 443
92
The average concentration of hemoglobin in a given volume of packed red cells, expressed in g/dL.
Mean cell hemoglobin concentration (MCHC)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
93
Renal effects of multiple myeloma
1) Formation of proteinaceous casts, one component of which are Bence Jones proteins, which can be toxic to the tubules. 2) Metastatic calcification. 3) Light chain amyloidosis. 4) Bacterial pyelonephritis due to susceptibility to infections (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 439
94
Hodgkin lymphoma subgroup with highest count of RS cells.
Mixed cellularity type(TOPNOTCH)
95
Deficiency in Vitamin B12 causes this type of anemia.
Pernicious anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
96
T Cells with abundant cytoplasm 12-16um in diameter that contains azurophilic granules, and an oval, indented or folded nucleus. SEE SLIDE 12.9
Atypical lymphocytes (Infectious mononucleosis)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 442
97
A 2 y/o child arrived at well-child clinic. Mother reported that her child is exclusively breastfed for 6 months and then supplemented mainly by carrots. The patient is most likely prone to developing anemia caused by:
Vitamin B12 deficiency (TOPNOTCH)
98
Treatment for sickle cell disease by increasing levels of HbF.
Hydroxyurea(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
99
In this disease, the marrow is populated by abnormal precursors-- megaloblastoid erythroid precursors, ringed sideroblasts, and granulocyte precursors with abnormal granules. SEE SLIDE 12.26
Myelodysplastic Syndrome (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 445
100
Represents a sudden but usually temporary cessation of erythropoeisis, usually trigerred by parvovirus B19 infections in patients with sickle cell disease.
Aplastic crises(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
101
Disease caused by deletion of 3 alpha globin genes.
Hemoglobin H disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
102
A 28 year old female is found to have a mediastinal mass on chest xray during a preemployment medical exam. On history, she is found to have muscle fatigue that worsens as the day progresses. She undergoes surgery where her mediastinal mass is resected. Microscopic examination showed sheets of bland spindle cells with sparse inflammatory infiltrates. SEE SLIDE 12.25. Her tumor is (A) a metastasis from an undiagnosed endometrial mass (B) a metastasis from an occult breast malignancy (C) a lymphoma(D) a thymoma
a thymoma (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 476
103
Two major consequences that arise from the sickling of red cells in sickle cell disease.
Chronic hemolytic anemia (Episodes of sickling damage the membrane. Mean life span is only 20 days) and Microvascular obstruction (Pain crises and hypoxic damage) (TOPNOTCH) Robbins Basic Pathology, 9th ed., p 412
104
Red cells with a central, dark-red puddle due to collection of hemoglobin.
Target cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
105
Small, dark nuclear remnants seen within red cells in PBS of hereditary spherocytosis. SEE SLIDE 12.2
Howell-Jolly bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
106
Excess light or heavy chains along with complete Igs synthesized by neoplastic plasma cells.
Bence-Jones proteins(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 454
107
Most common lymphoma of adults
Diffuse Large B-Cell Lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
108
Decreased gp IIb-IIIa leads to defective platelet aggregation, associated with normal platelet count.
Glanzmann thrombasthenia(TOPNOTCH)
109
Tumor of the thymus associated with myasthenia gravis and pure red cell aplasia.
Thymoma(TOPNOTCH)
110
Type of lymph node hyperplasia characterized by 1) preserved LN architecture, 2) variation in size of germinal centers, 3) presence of lymphocytes and phagocytic macrophages (tingible body macrophages). Causes include RA, toxoplasmosis, early HIV infection.
Follicular hyperplasia(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 428
111
Leukemia of children most responsive to chemotherapy.
Acute Lymphoblastic Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 461
112
An 8 year old child from the slums has easy fatigability and pallor. A peripheral blood smear shows large, egg-shaped red cell precursors, and hypersegmented neutrophils. SEE SLIDE 12.7. Short of measuring serum folate and vitamin B12 levels, what finding will suggest a vitamin B12 deficiency, rather than a folate deficiency? (A) gastrointestinal symptoms (B) neurologic symptoms (C) development of congestive heart failure (D) megaloblasts on bone marrow aspirate smears
neurologic symptoms (TOPNOTCH)Robbins Basic Pathology, 8th Ed. Pp 437-439
113
Associated with childhood onset microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. No neurologic symptoms. Often with a history of bloody diarrhea.
Hemolytic uremic syndrome(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 454
114
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.
Myeloid metaplasia with Myelofibrosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
115
The leading cause of cancer deaths in children
ALL (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
116
An 8 month old presented with anemia. Red cells were noted to be oval and macrocytic. Nutritional history revealed that the infant was exclusively fed raw goat's milk. The most likely cause of his anemia is:
Folate deficiency (TOPNOTCH)
117
A 40 y/o male presented with unexplained weight loss, fever, and night sweats. Chest radiograph showed a mediastinal mass. Histologic findings showed large cells with multiple nuclei with large inclusion-like nucleolus. What is the most likely diagnosis?
Hodgkin Lymphoma (Presence of Reed-Sternberg cells) (TOPNOTCH)
118
Condition caused by deletion of 2 alpha globin genes.
Alpha thalassemia trait(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
119
An 18 y/o female complained of generalized weakness, lethargy, and light headedness. She revealed she was having excessive bleeding during menstruation from the previous 6 months. Upon examining, she was noted to have pallor, tachycardia, and swollen tongue. Most likely morphology of RBC:
Microcytic, hypochromic anemia (Case of IDA) (TOPNOTCH)
120
HODGKIN or NON-HODGKIN LYMPHOMA: Orderly spread by contiguity
HODGKIN (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 442
121
This results from substitution of valine for glutamic acid at the 6th position of the B-chain, producing HbS.
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 426
122
Virus implicated in acute red cell aplasia
Parvovirus B19 (TOPNOTCH)) Robbins Basic Pathology, 9th ed., p. 655
123
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.
Acute nonspecific lymphadenitis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
124
Peripheral smear reveals circulating neutrophils, metamyelocytes, and myelocytes. Leukocyte count often exceeding 100,000 cells/uL. SEE SLIDE 12.24
CML (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 446
125
Hodgin lymphoma subgroup not associated with EBV.
Nodular sclerosis and lymphocyte predominant(TOPNOTCH)
126
Major pathologic feature of multiple myeloma
Bone destruction mediated by neoplastic plasma cells(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 599
127
Cells with two mirror-image nuclei or nuclear lobes, each containing a large acidophilic nucleolus surrounded by a distinctive clear zone, imparting an owl-like appearance. The sine qua non of Hodgkin Lymphoma.
Reed-Sternberg cell. SEE SLIDE 12.15(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 440
128
Smudge cells are seen in this type of leukemia. SEE SLIDE 12.14
CLL/SLL. There is absolute lymphocytosis of MATURE-LOOKING lymphocytes, but they are very fragile, hence the smudge cells. (TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
129
A 33 year old female presents with pallor, easy fatigability, and echymoses. Her spleen is not enlarged. A CBC showed profound anemia and markedly decreased WBC and platelet counts. A bone marrow core biopsy showed marrow that is predominantly replaced by fat, with few lymphocytic and plasma cells. SEE SLIDE 12.8. The most common cause of her condition is (A) myelotoxic drugs (B) viral infection (C) bacterial infection (D) idiopathic
idiopathic (aplastic anemia) (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P439
130
Hodgkin lymphoma subgroup most commonly associated with EBV infection.
Lymphocyte depleted(TOPNOTCH)
131
The average content of hemoglobin per red cell
Mean cell hemoglobin (MCH)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
132
Associated with pentad of fever, thrombocytopenia, microangipathic hemolytic anemia, transient neurologic deficits and renal failure.
Thrombotic thrombocytic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
133
True or False. Cellular immunity is relatively unaffected in Multiple myeloma
True(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 600
134
A mutation in the gene PIGA causes this disease that makes red blood cells sensitive to complement-mediated lysis.
Paroxysmal nocturnal hemoglobinuria(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 417
135
In beta thalassemia, an individual who inherits one abnormal allele (out of 2) has this asymptomatic to mildly symptomatic condition.
B- Thalassemia minor/trait(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
136
Myeloid tumor arising from a pluripotent stem cell associated with mutatios of the BCR-ABL gene. If untreated, may progress to a blast crisis.
Chronic Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
137
Prominent cheekbones and changes in skull resembling a "crew-cut" skull x-ray.
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427
138
On PBS, red cells are spherical which lack central pallor, and they show increased osmotic fragility when placed in hypotonic salt solutions. SEE SLIDE 12.1
Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
139
Hemolysis which takes place largely within phagocytic cells of the spleen and liver.
Extravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
140
HODGKIN or NON-HODGKIN LYMPHOMA: Involvement of multiple peripheral nodes
NON-HODGKIN (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 442
141
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. SEE SLIDE 12.1
Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
142
Drug-induced disorder caused by IgG antibodies that bind to platelet factor IV on platelet surfaces, which activates platelets and induce their aggregation.
Heparin-Induced Thrombocytopenia (HIT)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
143
Patient with this disease has a tendency toward easy bruising and massive hemorrhage after trauma or operative procedures, and recurrent bleeding into the joints. Petechiae are absent. Patients have a prolonged PTT and normal PT.
Hemophilia A and B (Factor VIII and IX Ddeficiency) (TOPNOTCH) Robbins Basic Pathology, 9th ed. p. 663
144
This correlates with good prognosis in Hodgkin lymphoma.
High Lymphocyte : Reed-Sternberg cell ratio(TOPNOTCH)
145
Precipitates of denatured globin seen in RBC's. SEE SLIDE 12.4.
Heinz bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
146
Major complication of sickle cell disease which occurs in the setting of acute chest syndrome, causing ischemic injury to the CNS.
CNS stroke(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
147
Tear drop cells are also called __________.
Dacrocytes(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440
148
A 17 y/o male presented with a short history of fever, tonsillitis and monolateral enlarged cervical lymph nodes. PE revealed enlargement of righ cervical lymph node, 3 cm in diameter hard , and pharyngeal hyperemia. Biopsy was done and showed a malignant population of round monomorphic B cells interspersed with macrophages forming the start in the "starry sky" pattern. SEE SLIDE 12.13. This is a case of:
Burkitt lymphoma (TOPNOTCH)
149
Red blood cells in iron deficiency anemia.
Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 436
150
Decreased gp Ib leads to defective platelet adhesion, associated with decreased platelet count.
Bernard-Soulier Syndrome(TOPNOTCH)
151
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.
Paracortical hyperplasia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
152
Principal causes of megaloblastic anemia.
Folate deficiencyVitamin B12 deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
153
Bite cells are seen in ________. SEE SLIDE 12.5.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
154
Individuals with B-thalassemia who inherit two abnormal alleles, with severe anemia requiring regular blood tranfusions.
B- Thalassemia major(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
155
A circulating protein that binds and clears free hemoglobin.
Haptoglobin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
156
The most common cause of agranulocytosis
Drug toxicity (TOPNOTCH) Robbins Pathologic Basis of Disease, 9th ed., p. 582
157
A 5 year old male is observed by his mother to have tea-colored urine whenever he has infections. One particular episode of pneumonia required hospital admission, where the child developed jaundice also. A peripheral blood smear showed red cells with Heinz bodies, while other red cells had parts of their cytoplasm "plucked out". SEE SLIDES 12.4 and 12.5. The child likely has a deficient enzyme required in the production of reduced glutathione, the gene for which is found on (A) X chromosome (B) Y chromosome (C) chromosome 21 (D) chromosome 22
X chromosome (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 431-432
158
Anemia observed in a variety of pathologic states, in which small vessels become particularly obstructed.(e.g. DIC, malignant HTN, SLE, etc.)
Microangiopathic hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
159
Frequent small "cleaved" cells mixed with large cells, growth pattern is NODULAR, centroblasts present. SEE SLIDE 12.11. Occurs in older adults, usually involves nodes, marrow, spleen. Associated with t(14;18) that results in overexpression of cyclin D1.
Follicular lymphoma(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 443
160
A 19 y/o African-American male presented with severe pain in the chest and extremities, splenomegaly, and anemia. PBS showed reticulocytosis, presence of target cells, and sickled cells. Three days prior , patient developed cough and fever. For the past 3 years, patient had suffered from recurrent pains and jaundice. What is the pathophysiologic mechanism responsible for the most serious clinical features of this disease?
Microvascular occlusion (in Sickle cell disease) (TOPNOTCH)
161
Etiology of pernicious anemia.
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
162
Fragile neoplastic lymphocytes that are frequently disrupted during smear preparation.
Smudge cells. SEE SLIDE 12.14 (TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
163
A 14 year old male develops fever, sore throat, lymphadenitis, and fatigue. His CBC shows leukocytosis, with a lymphocytic predominance. Peripheral blood smear shows some large leukocytes with abundant cytoplasm occasional azurophilic granules, and indented nuclei with fine chromatin. Monospot test and anti-EBV titers are positive. The large leukocytes seen are (A) monocytes (B) megakaryocytes (C) B cells (D) T cells
T cells (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 443
164
Anemia of beta thalassemia.
Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
165
Diagnostic criteria for iron deficiency anemia:____ Ferritin____Serum iron level____Transferrin saturation____Total Iron Binding Capacity (TIBC)
Low ferritinLow serum iron levelsLow transferrin saturationIncreased TIBC(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 435
166
Hemolysis that can result from mechanical trauma, or biochemical or physical agents that damage the red cell membrane.
Intravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
167
Caused by antiplatelet antibodies directed against glycoproteins IIb-IIIa, Ib-IX.
Immune thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
168
A 55 year old male presents with pallor and easy fatigability. Physical exam showed massive splenomegaly. CBC showed marked leukocytosis, and a peripheral smear showed numerous neutrophils, metamyelocytes, and myelocytes. SEE SLIDE 12.24. Basophils are also seen. A bone marrow aspirate shows a similar picture. This man likely has (A) a BCR-ABL fusion gene (B) a JAK2 mutation (C) an 8:22 translocation (D) an 11:22 translocation
a BCR-ABL fusion gene (Chronic myelogenous leukemia) (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 464
169
This lymphoma often presents a mass involving the mandible and shows an unusual predilection for involvement of abdominal viscera, particularly the kidneys, ovaries, and adrenal glands.
Endemic Burkitt lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 597
170
Cells found in adult T-cell lymphoma which appear to have multilobulated nuclei.
Cloverleaf or flower cell. SEE SLIDE 12.17. (TOPNOTCH)
171
Condition caused by deletion of 1 alpha globin gene.
Silent carrier(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
172
Caused by deficiency of ADAMTS13, a vWF metalloprotease.
Thrombotic thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
173
LYMPHOBLAST or MYELOBLAST: Often peroxidase positive
Myeloblast (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 433
174
Disease caused by Bartonella henselae, which morphologically presents as irregular stellate necrotizing granulomas in the nodes.
Cat scratch disease(TOPNOTCH)Robbins Basic Pathology, 9th ed., p 428
175
Polycythemia secondary to reduced plasma volume.
Relative polycythemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
176
Polycythemia secondary to abnormal proliferation of myeloid stem cells and low erythropoeitin levels.
Primary polycythemia (Absolute)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
177
Presence of anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, and jaundice are manifestations of intravascular or extravascular hemolysis?
Intravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 631
178
PBS finding in neutrophils and red cells of patients with megaloblastic anemia.
Hypersegmented neutrophils, large, egg-shaped macro-ovalocytes. SEE SLIDE 12.7 (TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
179
Other name for acute disseminated Langerhans cell histiocytosis.
Letterer-Siwe disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 468
180
Cells with blue globular nuclear inclusions, seen in Multiple myeloma
Dutcher bodies. SEE SLIDE 12.20 (TOPNOTCH)
181
Lymph nodes that drain cancers but do not yet harbor metastatic deposits often show (A) follicular hyperplasia (B) paracortical hyperplasia (C) sinus histiocytosis (D) fibrous obliteration
sinus histiocytosis (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 444
182
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. Reed Sternberg cells are uncommon. There are also collagen bands that divide the tissue into circumscribed nodules, hence the name. SEE SLIDE 12.16.
Nodular sclerosis HL(TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 441
183
X-linked disorder in which red cells are unusually susceptible to damage cause by oxidants. (Drugs that produce oxidants include antimalarials, sulfonamides, nitrofurantoin, phenacetin, and vitamin K derivatives)
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
184
Structural proteins that are defective in hereditary spherocytosis.
Spectrin and ankyrin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
185
The immediate cause of megaloblastosis, and a common denominator of folic acid and vitamin B12 deficiency
Suppressed synthesis of DNA (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 648
186
Most common form of HL in patients greater than 50 years old, with male predominance, plentiful RS cells and heterogenous cellular infiltrates.
Mixed cellularity HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 458
187
Most common bleeding disorder.
vWF disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
188
Hemolysis caused by IgG or IgA antiodies that are active at 37 degC, which results in opsonization of red cells by the autoantibodies.
Warm antibody immunohemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
189
Collection of aggressive tumors that are comprised of immature myeloblasts which replace the marrow and suppress normal hematopoiesis.
Acute Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
190
HODGKIN or NON-HODGKIN LYMPHOMA: Mesenteric nodes and Waldeyer ring are commonly involved
NON-HODGKIN (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 442
