XII - The Hematopoietic and Lymphoid Systems Flashcards
True or False. Age younger 2 years is associated with a worse prognosis in ALL.
True(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
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
Hodgkin lymphoma subgroup with poorest prognosis.
Lymphocyte depleted HL(TOPNOTCH)
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 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
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.
Acute lymphocytic leukemia (ALL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 447
t(9;22) is also called ______.
Philadelphia chromosome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 465
LYMPHOBLAST or MYELOBLAST: More cytoplasm, which often contains granules
Myeloblast (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 433
Type of lymph node hyperplasia characterized by distention of sinusoids due to macrophage infiltration and hypertrophy of endothelial cells. Encountered in cancer.
Sinus histiocytosis (TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 428
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
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
Single most important factor in the pathogenesis of renal failure in Multiple myeloma
Bence-Jones proteinuria(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 600
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)
Most common nutritional disorder in the world
Iron Deficiency Anemia(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 649
The most common trigger for episodic hemolysis in G6PD Deficiency
Infection(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 634
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)
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
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
What laboratory finding differentiate leukemoid reaction from CML?
Elevated leukocyte alkaline phosphatase. (TOPNOTCH)
Bizarre, elongated, spindled or boat-shaped cells on PBS. SEE SLIDE 12.3
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427
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 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)
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
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
Anemia of acute blood loss is described as ______.
Normocytic, normochromic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
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
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
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)
A reduction in the oxygen-transporting capacity of blood.
Anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 422
Increase in blood concentration of red cells, with an increase in Hgb concentration.
Polycythemia or erythrocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
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)
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)
Target cells are often seen in this condition.
B-thalassemia minor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
Most common etiology of aplastic anemia
Idiopathic (TOPNOTCH)Robbins Basic Pathology, 9th Ed. p. 424
Difference between megaloblastic and pernicious anemia.
Presence of neurologic abnormalities in pernicious anemia.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
The coefficient of variation of red cell volume.
Red cell distribution width (RDW)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
LYMPHOBLAST or MYELOBLAST: Often periodic acid-Schiff (PAS) positive
Lymphoblast (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 433
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
Treatment for sickle cell disease by increasing levels of HbF.
Hydroxyurea(TOPNOTCH)
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
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
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
Two major mechanisms that trigger DIC
Release of tissue factor and widespread endothelial injury(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 663
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
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
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
Hemolysis and vaso-occlusive crisis are common in this form of anemia caused by mutaion of glutamic acid to valine.
Sickle-cell anemia(TOPNOTCH)
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
Causative agent for cat scratch disease(TOPNOTCH)
Bartonella henselae(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
Most feared complication of thrombocytopenia
Intracranial bleeding (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 657
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
HODGKIN or NON-HODGKIN LYMPHOMA: Extranodal involvement common
NON-HODGKIN (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 442
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
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
Antibody that can be detected in EBV infection, which is used as a diagnostic test
Heterophil antibody (TOPNOTCH) Robbins Basic Pathology, 9th ed 426
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)
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
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
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
Leading cause of disease-related death in individuals with PNH
Thrombosis(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 642
HODGKIN or NON-HODGKIN LYMPHOMA: Often localized to a single axial group of nodes
HODGKIN (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 442
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
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
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
LYMPHOBLAST or MYELOBLAST: Coarse and clumped chromatin with scant cytoplasm
Lymphoblast (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 433
Cells with pink globular cytoplasmic inclusions, seen in Multiple myeloma.
Russell bodies. SEE SLIDE 12.20 (TOPNOTCH)
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
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
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
Condition caused by deletion of all four alpha globin genes.
Hydrops fetalis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
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
Characterized by spontaneous bleeding, prolonged bleeding time, and normal PT and PTT.
Thrombocytopenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 471
Principal neurologic lesion in pernicious anemia.
Demyelination of posterior and lateral columns of the spinal cord.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439
Dacrocytes are found in peripheral blood of patients with this type of anemia.
Myelophthisic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440
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
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