XII - The Hematopoietic and Lymphoid Systems Flashcards
Average volume per red blood cell
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
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. 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
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. SEE SLIDE 12.1
Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
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
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
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. SEE SLIDE 12.3
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. SEE SLIDE 12.4.
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 ________. SEE SLIDE 12.5.
G6PD Deficiency(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. SEE SLIDE 12.6
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. (Drugs that produce oxidants include antimalarials, sulfonamides, nitrofurantoin, phenacetin, and vitamin K derivatives)
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. SEE SLIDE 12.7 (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. SEE SLIDE 12.8
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
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
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
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
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. 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
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
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
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. 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
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
Fragile neoplastic lymphocytes that are frequently disrupted during smear preparation.
Smudge cells. SEE SLIDE 12.14 (TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
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
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