Hemapoetic and Lymphoid Systems

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  1. Inherited defect in RBC membrane with a deficiency of spectrin
    Hereditary spherocytosis
  2. Blood smear shows Reticulocytosis in peripheral blood
    Hereditary spherocytosis
  3. Splenomegaly, cholelithiasis, anemia
    hereditary spherocytosis
  4. Hereditary spherocytosis tx
    Splenectomy to correct anemia, but red cell defect persists
  5. Mutation in the gene coding for a b –globin chain
  6. What is the difference between a homozygote and heterozygote in SCA?
    • Homo: all Hb Is replaced by Hbs
    • Hetero: One half is replaced by hemoglobin S
  7. Most common form of familial hemolytic anemia worldwide
  8. What determines severity of SCA?
    Amount of HbS
  9. Microvascular obstructions put people with SCA at risk for what?
  10. Substitution of valine for glutaminic acid on b chain
  11. What are signs and symptoms of SCA?
    • Fatty changes in the heart, liver, and renal tubules
    • Erythropoesis with new bone formation due to hemolytic anemia
    • Initial splenomegaly followed by autosplenectomy
    • Vascular congestion, thrombosis, infarction
  12. Describe vasoocclusive crisis in sickle cell
    Something will trigger the dehydration and sickle crisis --> pain in abdomen, joints, chest which is precipitated by dehydration, infection or acidosis
  13. cessation of erythropoiesis
    anaplastic crisis (associated with SCA)
  14. Lack of or decreased synthesis of globin chains
  15. Genetic cause of beta thalassemia
    Single base changes as opposed to gene deletions
  16. Why are beta thalassemia cells hypochromic?
    reduced synthesis of beta-globin causes inadequate HbA formation
  17. What causes hemolysis in beta thalassemia?
    Excess of alpha globin chains precipitating and causing phagocytosis
  18. Why does iron absorption increase in beta thalassemia?
    body things there is an IDA
  19. Due to deletions of alpha globin gene loci
    Alpha thalassemia
  20. Difference in hemolysis between beta and alpha thalassemia
    Alpha hemolysis is less severe
  21. Microcytic hypochromic red cells
  22. Poikilocytosis, anisocytosis, reticulocytosis
  23. hair on end appearance
  24. Secondary hemochromotosis
  25. What is the prognosis of thalassemia major?
    need blood transfusions, develop secondary hemochromatosis, average age of death is 17
  26. What is the prognosis of thalassemia minor?
    mild microcytic hypochromic anemia with a nl life expectancy
  27. Heinz bodies
  28. What causes G6PD deficiency?
    Lack of GSH leading to oxidation injury
  29. Chronic intravascular hemolysis
    Paroxysmal Nocturnal Hemoglobinuria
  30. Membrane defect from mutation affecting myeloid stem cells
    Paroxysmal Nocturnal Hemoglobinuria
  31. Paroxysmal Nocturnal Hemoglobinuria has a risk of what?
    progression to leukemia
  32. Yields RBCs, granulocytes, and platelets that are very sensitive to lytic activity of complement
    Paroxysmal Nocturnal Hemoglobinuria
  33. pts RBCs coated with human antibodies that react with anti human globulin serum
    Direct Coombs test associated with immunohemolytic anemia
  34. Used to detect antibodies in pts serum, incubate normal RBCS with pts serum followed by a direct Coombs test on the incubated RBC
    Indirect COombs test associated with immunohemolytic anemia
  35. Opsonization of rbc by IgG and phagocytosis by splenic macrophages
    • Warm antibody immunohemolytic anemia
    • Activated at 37C
    • Primary 60%, secondary to lymphoma, SLE, drugs
    • Splenomegaly
  36. Cold antibody immunohemolytic anemias
    • igM bindes to RBC at temp below 37C
    • Cooler parts of the body (fingers, toes)
    • RBC sequestered in the liver
    • Hemolysis is extravascular, usually in spleen
  37. What are causes of mechanical hemolysis?
    Prosthetic cardiac valves, atherosclerosis, DIC [RBCs hit thrombi and becomes sheared]
  38. Hemolytic anemia associated with high fever, HA, sweating, delerium
  39. M/C nutritional deficiency
  40. low hb ,low hematocrit, low mcv,low transferin saturation, increased total iron binding capacity
  41. Plummer-Vinson syndrome
  42. IDA is what kind of anemia?
    microcytic hypochromic
  43. Decline in serum ferritin and stainable iron in bone marrow
  44. What conditions can cause anemia of chronic disease?
    Osteomyelitis, endocaritis, RA, HL, lung and breast Ca
  45. What is the biggest difference between IDA and anemia of chronic dz?
    Anemia of chronic dz has a reduced total iron-binding capacity, while it is increased in IDA
  46. Abundant stored iron in mononuclear phagocytic cells as well as high serum ferritin
    Anemia of chronic dz
  47. What are the two types of megaloblastic anemias?
    Folate deficiency and pernicious anemia
  48. enlarged erythroid precursors accumulate in bm yielding too few erythrocytes
    Megaloblastic anemia
  49. What do you see in the peripheral blood of someone with megaloblastic anemia?
    Large oval erythrocytes, hypersegmented granulocytes, and large mishapen platelets
  50. Fatigue , Weakness, Cheilosis, Sore tongue, Neurologic abnormalities absent
    Folate deficiency anemia
  51. Cause of pernicious anemia
    Usually from inadequate production of or function of intrinsic factor, appears to be an autoimmune dz
  52. Describe the autoimmune nature of pernicious anemia
    • Autoantibodies block binding of if to b12
    • Autoantibody binds to if and ifb12 complex
    • Autoantibody binds to parietal cell border
  53. What procedures can cause pernicious anemia?
    • Gastrectomy,
    • resection of ileum
    • tropical sprue
    • Whipple's disease
  54. Demyelination of posterior and lateral columns of spinal cord
    Pernicious anemia
  55. Numbness ,tingling, unsteadiness of gait
    Fatigue ,pallor
    Pernicious anemia
  56. Pts with pernicious anemia are at an increased risk for what?
    gastric cancer
  57. What test helps you differentiate between pernicious anemia and folate defeciency?
    Schilling test: inability to absorb oral dose of cobalamin
  58. shutting down production of RBCs, neutrophils and platelets
    Aplastic anemia
  59. Normochromic normocytic anemia, Neutropenia,
    aplastic anemia
  60. What are causes of aplastic anemia?
    • Metastatic Ca
    • Liver dz, chronic renal dz
    • Whole body irradiation or chemo
  61. Absolute increase in red cell mass and low erythropoetin level
    P vera
  62. Hypercellular bone marrow with proliferation of granulocytic and megakaryocytic and megakaryocytic elements
    P vera
  63. Long term survivors may undergo transition to myeloid metaplasia with myelofibrosis
    P vera
  64. What are the two causes of neutropenia agranulocytosis?
    • Inadequate granulopoiesis (drugs)
    • Accelerated destruction of neutrophils (drugs, idiopathic)
  65. When do you see PMNs?
    Bacteria, tissue necrosis (infarctions)
  66. When do you see eosinophils?
    Parasites, allergies, drug rxs
  67. When do you see monocytess?
    Chronic infections, collage vascular dz
  68. When do you see lymphocytes?
    viral infections
  69. Bartonella henselae
    Cat scratch dz
  70. Granulomas with central necrosis
    cat scratch dz
  71. How does cat scratch dz present?
    Lymphadenopathy of the head and neck 2 weeks after the scratch; regresses within 2-4 mo
  72. Where can leukemic cells infiltrate?
    Blood, liver, spleen, and LN
  73. Anemia,fever,bleeding, easy bruising, fatigue, Bone pain and tenderness
    Acute leukemia
  74. Cns: ha,nerve palsies, vomiting
    Acute leukemia
  75. What is the age difference between the acute leukemias?
    • Acute lymphoblastic: <15, peak 4 yo
    • Acute myeloblastic: mostly adults, med age 50 years
  76. red rod shaped lysosomes
    Auer rods, seen in myelblastic leukemias
  77. What is the age difference between the chronic leukemias?
    • Chronic myeloid: 25-60
    • Chronic lymphoid over age 50
  78. White counts usually > 100k; massive splenomegaly
    chronic myeloid leukemia
  79. Circulating cells mostly neutrophils and myelocytes
    chronic myeloid leukemia
  80. Leukemic cells have decreased alkaline phosphatase activity
  81. 2-3 year course without tx; Tx induces 2-5 year remission followed by blast crises and death
  82. What is the only cure for CML?
    BM transplant
  83. Nonfunctioning B cells produce a hypogammaglobulinemia – no production of plasma cells
  84. May have an autoimmune hemolytic anemia
  85. Median survival is 4-6 years; Pts may live over 10 years
  86. Leukemic cells have fine hair-like cytoplasmic projections
    Hairy cell leukemia
  87. Tartrate resistant acid phosphatase in neoplastic B cells
    Hairy cell leukemia
  88. Leukemia affecting Older males, insidious onset characterized by splenomegaly and pancytopenia
    Hairy cell leukemia
  89. Cells have Birbeck granules in the cytoplasm
    Langerhan's cell histiocytosis
  90. What are the three components of Langerhan's cell histiocytosis?
    • Letter-Siwe dz
    • Unifocal Langerhan's cell histiocytosis
    • Multifocal Langerhan's cell histiocytosis
  91. What age group does Letter-Siwe affect?
    Before two years old
  92. What are characteristics of Letter-Siwe?
    Skin lesions, H-S megaly, lympadenopathy, pulmonary lesions, bone lesions, anemia and infections
  93. Rapidly fatal without chemotherapy
  94. Expansive erosive lesions in bones with siilar lesions found in skin, lungs, or stomach
    Unifocal Langerhan's cell histiocytosis
  95. What is the Hand-Schuller-Christian triad?
    Calavarial bone defects, diabetes insipidus, and exophthalmos (associated with multifocal Langerhan's cell)
  96. OM, mastoiditis, URI infections with lesions on the scalp and ear canal
    Multifocal Langerhan's cell
  97. tests for extrinsic and common coagulation pathways
  98. evaluates the common and intrinsic clotting pathways
  99. What can cause a prolonged PT time?
    deficiency of factors V,VII, or X, prothrombin or fibrinogen
  100. What can cause an increased PTT time?
    deficiency of factors V,VIII,IX,X,XII, prothrombin or fibrinogen
  101. What is DIC secondary to?
    Sepsis, prolonged deliveries, fat or amniotic fluid emboli
  102. Activation of coagulation sequence: Excessive clotting at first and using up platelets and coagulation fa
  103. What are pathological steps of DIC?
    • Consumption of plts and coag factors
    • Activation of fibrinolysis
    • Tissue infarcts and hypoxia
    • Bleeding DO
  104. What is the difference between acute and chronic DIC?
    • acute: bleeding DO
    • chronic: thrombotic complications
  105. Spontaneous bleeding, Prolonged bleeding time, with nl PT/PTT
  106. What causes thrombocytopenia?
    • Decreased production of plts: BM disease
    • Decreased plt survival: immunologic and nonimmunologic destruction
  107. Bleeding disorder that is one of the most common manifestations of AIDs
  108. Idiopathic thrombocytopenic purpura tends to affect who?
    Adults and females
  109. Production of antibodies against plts in the spleen
    Idiopathic thrombocytopenia purpura
  110. Hemorrhages in skin, epicardium, GIT,UT
    Idiopathic thrombocytopenia purpura
  111. How do you tx idiopathic thrombocytopenic purpura?
  112. What population does thrombotic thrombocytopenic pupura tend to affect?
    Adult females
  113. Bleeding DO with transient neurological deficits: Can mimic stroke, MS, bells palsy
    Thrombotic thrombocytopenic purpura
  114. What is the biggest complication people with thrombotic thrombocytopenia face?
    Renal failure
  115. Thrombocytopenia with microangiopathic
    hemolytic anemia but no neurologic symptoms
    Hemolytic uremic syndrome
  116. Hemolytic uremic syndrome usually follow what?
    E coli gastereneritis
  117. When is Hemolytic uremic syndrome's onset?
  118. Spontaneous bleeding from mucous membranes-usually not a lot of bleeding
    Von Willebrand Disease
  119. Menorrhagia, prolonged bleeding time, nl plt count
    Von Willebrand Disease
  120. What causes Von Willebrand Disease?
    compound defect of plt function and coag pathway
  121. Most common hereditary disease associated with serious bleeding
    Hemophilia A
  122. Reduced amount or reduced activity of factor VII
    Hemphilia A
  123. X linked, usually in males
    Hemophilia A
  124. What are lab levels in Hemophilia A?
    Nl PT, PTT, platelet counts
  125. How do you treat Hemophilia A?
    Infusion of factor VIII
  126. Factor IX deficieny
    Hemphillia B
  127. Which Hemophilia is more common?
  128. What are the two major differences between Hemophilia A and B?
    • A is Factor 8 deficiency, B is factor 9
    • B has prolonged PTT while A is nl
  129. Appearance of lymphoid follicles in the medulla and seen in MG, SLE, RA
    Thymic hyperplasia
  130. Tumors with a neoplastic epithelial component
  131. What are the three kinds of thymomas?
    • Benign thymoma: cytologically and biologically benign
    • Malignant thymoma I :cytologically benign but biologically malignant
    • Malignant thymoma II: cytologically and behaviorly malignant
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Hemapoetic and Lymphoid Systems
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