XII - The Hematopoietic and Lymphoid Systems Flashcards
Average volume per cell, expressed in femtoliters.
Mean cell volume (MCV)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
A reduction in the oxygen-transporting capacity of blood.
Anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 422
The average content of hemoglobin per red cell, expressed in picograms.
Mean cell hemoglobin (MCH)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
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
The coefficient of variation of red cell volume.
Red cell distribution width (RDW)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
Anemia of acute blood loss is described as ______.
Normocytic, normochromic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
Life span of a normal red cell.
120 days(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
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.
Hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
A circulating protein that binds and clears free hemoglobin.
Haptoglobin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
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
Hemolysis which takes place largely within phagocytic cells of the spleen and liver.
Extravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
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.
Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
Small, dark nuclear remnants seen within red cells in PBS of hereditary spherocytosis.
Howell-Jolly bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
On PBS, red cells are spherical which lack central pallor, and they show increased osmotic fragility when placed in hypotonic salt solutions.
Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
Structural proteins that are defective in hereditary spherocytosis.
Spectrin and ankyrin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
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
Bizarre, elongated, spindled or boat-shaped cells on PBS.
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427
Prominent cheekbones and changes in skull resembling a “crew-cut” skull x-ray.
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427
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
Treatment for sickle cell disease by increasing levels of HbF.
Hydroxyurea(TOPNOTCH)
Treatment for sickle cell disease by increasing levels of HbF.
Hydroxyurea(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
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
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
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
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
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
Red cells with a central, dark-red puddle due to collection of hemoglobin.
Target cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
Target cells are often seen in this condition.
B-thalassemia minor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
In the PBS of this condition, nucleated red cells (normoblasts) are seen, which reflect underlying erythropoeisis.
B-thalassemia major(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
Anemia of beta thalassemia.
Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
Disease caused by deletion of 3 alpha globin genes.
Hemoglobin H disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
Condition caused by deletion of 1 alpha globin gene.
Silent carrier(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
Condition caused by deletion of 2 alpha globin genes.
Alpha thalassemia trait(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
Condition caused by deletion of all four alpha globin genes.
Hydrops fetalis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
Precipitates of denatured globin seen in RBC’s.
Heinz bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
Heinz bodies are seen in the blood smear of this condition.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
Bite cells are seen in ________.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
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.
Paroxysmal nocturnal hemoglobinuria(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
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
Anemia caused by low-affinity IgM which bind to red cell membranes only at temp
Cold antibody immunohemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
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
Schistiocytes, burr cells, helmet cells, triangle cells are seen in this condition.
Microangiopathic hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
X-linked disorder in which red cells are unusually susceptible to damage cause by oxidants.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
Red blood cells in iron deficiency anemia.
Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 436
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
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
Principal causes of megaloblastic anemia.
Folate deficiencyVitamin B12 deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
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
PBS finding in neutrophils and red cells of patients with megaloblastic anemia.
Hypersegmented neutrophils, large, egg-shaped macro-ovalocytes(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
Difference between megaloblastic and pernicious anemia.
Presence of neurologic abnormalities in pernicious anemia.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
Deficiency in folate causes this type of anemia.
Megaloblastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
Deficiency in Vitamin B12 causes this type of anemia.
Pernicious anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
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
Principal neurologic lesion in pernicious anemia.
Demyelination of posterior and lateral columns of the spinal cord.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439
Bone marrow is markedly hypocellular, with >90% of the intertrabecular space being occupied by fat.
Aplastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439
Tear drop cells are also called __________.
Dacrocytes(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440
Dacrocytes are found in peripheral blood of patients with this type of anemia.
Myelophthisic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440
Increase in blood concentration of red cells, with an increase in Hgb concentration.
Polycythemia or erythrocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
Polycythemia secondary to reduced plasma volume.
Relative polycythemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
Polycythemia secondary to abnormal proliferation of myeloid stem cells and low erythropoeitin levels.
Primary polycythemia (Absolute)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
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
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
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.
Infectious mononucleosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 442
Cells with abundant cytoplasm 12-16um in diameter that contains azurophilic granules, and an oval, indented or folded nucleus.
Atypical lymphocytes (Infectious mononucleosis)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 442
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
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.
Follicular hyperplasia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
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)
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
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.
Cat scratch disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
Causative agent for cat scratch disease(TOPNOTCH)
Bartonella henselae(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
Lymphoblasts with irregular nuclear contours, condensed chromatin , small nucleoli and scant agranular cytoplasm. Blasts compose >25% of marrow cellularity. Most common childhood leukemia.
Acute lymphocytic leukemia (ALL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 447
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).
Follicular lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 451
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).
Mantle cell lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 452
Plasma cells in sheets, with prominent nucleoli or inclusion containing Ig. Presents as disseminated bone disease, with destructive lytic lesions.
Plasmacytoma / plasma cell myeloma(TOPNOTCH)
Intermediate-sized round lymphoid cells with 2-5 prominent nucleoli. Nuclear remnants phagocytosed by interspersed macrophages with abundant clear cytoplasm, “starry sky pattern”
Burkitt lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 453
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.
Small lymphocytic leukemia (SLL) / Chronic lymphocytic leukemia (CLL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
Fragile neoplastic lymphocytes that are frequently disrupted during smear preparation.
Smudge cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
Smudge cells are seen in this type of leukemia.
CLL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
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
Multifocal destructive bone lesions seen as punched-out defects in bone radiographs.
Multiple myeloma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 455
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
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.
Reed-Sternberg cell(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 456
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.
Hodgkin Lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 456
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.
Nodular sclerosis HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 457
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
Cells found in adult T-cell lymphoma which appear to have multilobulated nuclei.
Cloverleaf or flower cell(TOPNOTCH)
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.
Lymphocyte-predominance HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 458
Cells with fiery red cytoplasm, seen in Multiple myeloma
Flame cells(TOPNOTCH)
Cells with pink globular cytoplasmic inclusions, seen in Multiple myeloma
Russell bodies(TOPNOTCH)
Cells with blue globular nuclear inclusions, seen in Multiple myeloma
Dutcher bodies(TOPNOTCH)
Multiple nuclei, prominent nucleoli, and cytoplasmic droplets containing Ig.
Bizarre, multinucleated cells(TOPNOTCH)
Bizarre multinucleated cells, flame cells, Russel bodies and Dutcher bodies are all seen in what disease?
Multiple myeloma(TOPNOTCH)
Bone marrow aspirate shows hypercellular martow packed with myeloblasts and azurophilic needle-like material called Auer rods.
Acute Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 462
Leukemia of children most responsive to chemotherapy.
Acute Lymphoblastic Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 461
Hodgkin lymphoma subgroup most commonly associated with EBV infection.
Lymphocyte depleted(TOPNOTCH)
Mature B-cell tumor in the elderly, where cells have fine hair-like projections (hairy cells).
Hairy cell leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 459
t(9;22) is also called ______.
Philadelphia chromosome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 465
Hodgkin lymphoma subgroup with highest count of RS cells.
Mixed cellularity type(TOPNOTCH)
This correlates with good prognosis in Hodgkin lymphoma.
High Lymphocyte : Reed-Sternberg cell ratio(TOPNOTCH)
Hodgin lymphoma subgroup not associated with EBV.
Nodular sclerosis and lymphocyte predominant(TOPNOTCH)
Hodgkin lymphoma subgroup with poorest prognosis.
Lymphocyte depleted HL(TOPNOTCH)
Tumor of the thymus associated with myastheni gravis and pure red cell aplasia.
Thymoma(TOPNOTCH)
Pentalaminar tubules, often with a dilated terminal end (tennis racket-like appearance).
Birbeck granules(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
Proliferative disorder of the dendritic cells which has birbeck granules.
Langerhans Cell Histiocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
Pathology behind polycythemia vera.
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
Treatment for polycythemia vera.
Phlebotomy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 466
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
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
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
Other name for acute disseminated Langerhans cell histiocytosis.
Letterer-Siwe disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 468
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
Characterized by spontaneous bleeding, prolonged bleeding time, and normal PT and PTT.
Thrombocytopenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 471
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
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
Associated with childhood onset microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. No neurologic symptoms.
Hemolytic uremic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
Caused by antiplatelet antibodies directed against glycoproteins IIb-IIIa, Ib-IX.
Immune thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
Decreased gp Ib leads to defective platelet adhesion, associated with decreased platelet count.
Bernard-Soulier Syndrome(TOPNOTCH)
Caused by deficiency of ADAMTS13, a vWF metalloprotease.
Thrombotic thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
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
Decreased gp IIb-IIIa leads to defective platelet aggregation, associated with normal platelet count.
Glanzmann thrombasthenia(TOPNOTCH)
Most common bleeding disorder.
vWF disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
Most common hereditary disease associated with life threatening bleeding,
Hemophilia A(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
An X-linked recessive disorder caused by reduction in factor VII activity.
Classic Hemophilia/ Hemophilia A(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
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
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
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. This is a case of:
Burkitt lymphoma (TOPNOTCH)
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?
AML (TOPNOTCH)
The most common cause of agranulocytosis
Drug toxicity (TOPNOTCH) Robbins Pathologic Basis of Disease, 9th ed., p. 582
What laboratory finding differentiate leukemoid reaction from CML?
Elevated leukocyte alkaline phosphatase. (TOPNOTCH)
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)
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)
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
Most common leukemia of adults which usually presents with bone marrow and lymph node involvement.
Small lymphocytic lymphoma/CLL(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
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
Most common lymphoma of adults
Diffuse Large B-Cell Lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
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
Plasma cell neoplasm commonly associated with lytic bone lesions, pathologic fractures, chronic pain, hypercalcemia, renal failure, and acquired immune abnormalities.
Multiple Myeloma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 599
Major pathologic feature of multiple myeloma
Bone destruction mediated by neoplastic plasma cells(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 599
True or False. Cellular immunity is relatively unaffected in Multiple myeloma
True(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 600
Single most important factor in the pathogenesis of renal failure in Multiple myeloma
Bence-Jones proteinuria(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 600
True or False. Age younger 2 years is associated with a worse prognosis in ALL.
True(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
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
The leading cause of cancer deaths in children
ALL (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
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
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)
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?
CML (TOPNOTCH)
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
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)
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)
Hemolysis and vaso-occlusive crisis are common in this form of anemia caused by mutaion of glutamic acid to valine.
Sickle-cell anemia(TOPNOTCH)
The most common trigger for episodic hemolysis in G6PD Deficiency
Infection(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 634
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)
Leading cause of disease-related death in individuals with PNH
Thrombosis(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 642
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
Most common nutritional disorder in the world
Iron Deficiency Anemia(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 649
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)
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
Virus implicated in acute red cell aplasia
Parvovirus B19 (TOPNOTCH)) Robbins Basic Pathology, 9th ed., p. 655
Most feared complication of thrombocytopenia
Intracranial bleeding (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 657
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.
von Willebrand disease (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 662
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
Two major mechanisms that trigger DIC
Release of tissue factor and widespread endothelial injury(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 663
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”. 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
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. 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
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. 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
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
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
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. 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
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
neoplastic megakaryocytes (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 466
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. 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
