Hematology Exam 2

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  1. ET Essential Thrombocythemia
    Rare CMPD characterized by marked thrombocytosis and abnormal platelet functions.
  2. Rare CMPD with thrombocytosis and abnormal platelet function.
    ET Essential thrombocythemia
  3. ET Causes and symptoms:
    • Clonal multipotential stem cell, mostly megakaryocytes
    • Hypersensitivity of megakaryocytes to cytokines and thrombopoietin
    • Absence of identifiable causes of reactive thrombocytosis
    • Absence of Philadelphia chromosome
    • Hgb no higher than 13 g/dL
    • Absence of significant marrow fibrosis
    • Consumption of coag factors- Acute hemorrhage
    • Presence of stainable iron in BM
  4. Myelofibrosis With Myeloid Metaplasia symptoms
    • bone marrow fibrosis
    • proliferation of megakaryocytic and granulocytic cells
    • extramedullary hematopoiesis-splenomegaly
    • Chromosomal abnormalities (no philly)
  5. Bone Marrow fibrosis, splenomegaly, anemia
    Myelofibrosis with Myeloid Metaplasia
  6. Myofibrosis with myeloid metaplasia- smear signs
    • Teardrop RBCs
    • Normochromic normocytic anemia
    • Leukoerythroblastosis
    • Giant platelets
    • Many nRBCs
  7. Teardrop RBCs
    Normochromic normocytic anemia
    Leukoerythroblastosis
    Giant platelets
    Many nRBCs
    Myelofibrosis with myeloid metaplasia
  8. 7 Lymphoproliferative disorders
    • Chronic Lymphocytic Leukemia (CLL)
    • Hairy Cell Leukemia
    • Sezary Syndrome
    • Prolymphocytic Leukemia- younger lymphs
    • Hodgkin’s and Non- Hodgkin’s lymphoma
    • Multiple Myeloma
    • Waldenstrom’s Macroglobulinemia
  9. CLL Cause
    Clonal B proliferation-chromosomes 11, 12, and 13, especially trisomy 12
  10. Clonal B proliferation
    CLL
  11. CLL Signs
    • Lymphadenopathy
    • Many white counts are >100,000 x 109/L
    • Peripheral smear shows exclusively small lymphocytes intermixed with few lymphoblasts
    • Smudge cells
    • nRBCs and spherocytes may be present if autoimmune hemolytic
  12. Image Upload
    • CLL Fragile small lymphocytes
    • Smudge cells
  13. CLL Symptoms
    • Splenomegaly & Lymphadenopathy- lymphs accumulating in nodes and spleen
    • Anemia, Neutropenia, & Thrombocytopenia-Lymph production crowds out other cell proliferation
    • Autoimmune hemolytic anemia- caused by altered immune function
  14. Splenomegaly
    Lymphadenopathy
    Anemia
    Neutropenia
    Thrombocytopenia
    Autoimmune hemolytic anemia
    CLL
  15. Hairy Cell Leukemia Cause
    B-cell malignancy
  16. Hairy Cell Signs and diagnosis
    • Fragile mononuclear cell
    • Hair-like projections of the cytoplasm
    • Spongy appearance of chromatin
    • Stain with TRAP- tartrate-resistant acid phosphatase
    • Thrombocytopenia
    • Pancytopenia
    • Massive spleen
    • Infection
  17. Image Upload
    Hairy Cell Leukemia
  18. Sezary Syndrome Causes
    • T-cell lymphoma with mycosis fungoides
    • Infiltration of Lymphocytes on skin
  19. Sezary Cell Description
    • Large cells (8 - 20 mm)
    • Ovoid, convoluted, cerebriform nucleus
    • May be mistaken for monocytes
    • Folded, brain-like
    • Clefted
    • Clover pattern
    • Blue cytoplasm
  20. Image Upload
    Sezary Syndrome-cutatneous T-cell lymphoma
  21. Prolymphocytic Leukemia (PLL)- Causes
    Variant of chronic lymphocytic leukemia
  22. PLL- Signs/Symptoms
    • Splenic enlargement
    • Liver involvement
    • Escalating white counts
    • Prolymphocytes have more abundant cytoplasm than mature lymphocytes
  23. Image Upload
    PLL
  24. Hodgkin’s Lymphoma-Causes
    People exposed to EBV or environmental hazards are at increased risk.
  25. Hodgekin's Lymphoma Signs/Symptoms/Diagnosis
    • Single cervical lymph node becomes firm to the touch and does not disappear
    • Low-grade fever
    • Weight loss
    • In lymph nodes - Presence of Reed-Sternberg cell
    • Diagnosis via lymph biopsy
  26. Image Upload
    • Reed-Sternberg Cell
    • Hodgekin's Lymphoma
  27. Reed-Sternberg Cell in Lymph nodes
    Hypermetabolism
    Cervical lymph node swollen
    Hodgekin's Lymphoma
  28. Non-Hodgkin’s Lymphoma Causes/Symptoms
    • May present as painless cervical lymph node involvement
    • Lymphoma cells may be seen in peripheral smear
    • Disease can spread
  29. Image Upload
    • Plasma Cells-Evolve from B-lymphocytes
    • Medium-sized cell
    • Have an eccentric nucleus
    • Have well-defined Golgi apparatus
    • Cytoplasm has distinct sea blue/cornflower color
    • Chromatin organized in a pinwheel structure
    • Make immunoglobulins
  30. Multiple Myeloma Cause
    • Disorder of Plasma Cells
    • Accumulation of plasma cells in BM and other locations
    • Exposure to atomic radiation
    • Work involving organic solvents
    • Toxins within the textile industry
    • Occupations with exposure to chemicals, pesticides, or herbicides
    • Chromosome 13 abnormalities
    • Caused by cytokine IL-6
  31. Disorder of Plasma Cells
    Multiple Myeloma
  32. 3 pathways of multiple myeloma
    • Acceleration of plasma cells in the bone marrow
    • Activation of bone resorption factors or osteoclasts
    • Production of an abnormal monoclonal protein
  33. Mulitple Myeloma Signs
    • Inclusions called Russell bodies or other crystalline inclusions
    • Flame cells may be seen in IgA myeloma -Deep pink cytoplasm
    • Monoclonal gammopathy
    • Increased osteoclast activity -Serum calcium increased
    • Rouleaux formation
    • Bence-Jones protein found in urine of patients with multiple myeloma
    • ESR usually elevated due to increased settling
    • "Punched-out” lesions on initial radiography
  34. Image Upload
    Russell Body in Multiple Myeloma
  35. Multiple Meyloma Symptoms
    • Anemia
    • Thrombocytopenia
    • Neutropenia
    • Can Spread in body as plasmacytomas
    • Fatigue
    • Bone Fractures
  36. Waldenstrom’s Macroglobulinemia Cause
    Overproduction of IgM caused by abnormal B lymphocytes
  37. Waldenstrom’s Macroglobulinemia
    Signs/Symptoms
    • IgM interferes with coagulation factors by coating platelets and impeding their function
    • Peripheral smear may show rouleaux and plasmacytoid lymphocytes
    • Cryoglobulins-May lead to Raynaud’s phenomenon(Cold, tingeling, fingers and toes); Causes bleeding; Cold agglutenins
  38. RBC Maturation Series
    • Pronormoblast
    • Basophilic Normoblast
    • Polychromatophilic normoblast
    • Orthochromic normoblast (NRBC)
    • Reticulocyte (requires stain)
    • Erythrocyte
  39. Secondary RBC Maturation Series
    • Rubriblast
    • Prorubricyte
    • Rubricyte
    • Metarubricyte
    • Diffusely Basophilic Erythrocyte
    • Erythrocyte
  40. Image Upload
    Target Cells
  41. Target Cells
    Excessive plasma chol. can lead to excessive membrane chol. and cause cell to become heavier and thicker, displacing the Hgb. Surface area to volume ratio increases, cell is less fragile.
  42. RBC Membrane protiens
    • Glycophorin A (Glycophorins are proteins
    • containing carbohydrates)
    • Spectrin
    • Ankyrin
  43. Image Upload
    Spherocytes
  44. 3 RBC Metabolism Pathways
    • Embden-Meyerof
    • Hexose Monophosphate Shunt
    • Methemoglobin Reductase
  45. Embden-Meyerhoff Pathway
    • Glycolytic
    • Generates 2,3 diphosphoglycerate-unloading of Oxygen
    • 90% of cell metabolism
    • Anareobic
  46. Generates 2,3-DPG
    • Embden-Meyerhoff pathway
    • Aids in unloading of oxygen
  47. Hexose Monophosphate Shunt
    • Produces NADPH
    • Protects against oxidative damage
  48. Produces NADPH, Protects against oxidative damage
    Hexose Monophosphate shunt
  49. If Hexose Monophosphate Shunt inaccurate:
    • Then - Heinz Bodies
    • Bite Cells
    • Cause- G6PD deficiency
  50. Heinz Bodies
    Caused by Denaturing of Hgb- precipitation in form of Heinz Bodies. HMS-G6PD deficiency
  51. Image Upload
    Heinz Bodies
  52. Image Upload
    Bite Cell
  53. Methemoglobin Reductase Pathway
    • Keeps heme in ferrous (+2) state
    • If deficient, cannot bind oxygen
  54. RBC Size
    • –Normal 6-8µm
    • –MCV 80- 100 fL
    • –MCHC 32% -36%
  55. Microcytic Anemias
    • Iron Def Anemia -IDA
    • Anemia of Inflammation-AoI
    • Thalassemia
    • Iron Overload Disorders
  56. Macrocytic Anemias
    • Megaloblastic-Folic acid or Vit. B-12 def
    • Liver Disease
    • MCV > 110fL
  57. Polychromasia
    • Gray/Blue Color
    • Retic released - BM response to anemic stress
  58. Image Upload
    Polychromatic RBC
  59. MCV
    • Mean Corpuscular Volume
    • 80-100fL, HCT/RBC x10
  60. MCH
    • Mean Corpuscular Hgb
    • 27-31pg
    • Hgb/RBC x10
  61. MCHC
    • Mean Corpuscular Hgb Concentration
    • 32-36%
    • Hgb/HTC x100
  62. RDW
    • RedCell Distribution Width
    • 11.5-14.5%
    • Anisocytosis
  63. Hgb
    • 14-18 Males
    • 12-16 Females
    • g/dL
  64. HCT
    • 42-52% Males
    • 37-47% Females
  65. WBC Count
    4-11 x10^9g/L
  66. RBC Count
    • 4.7-6.1 Males
    • 4.2-5.4 Females
    • x10^12/L
  67. RBC Color Classification
    • MCHC
    • Normochromic- 32-36%
    • Hypochromic- <32%
  68. RBC Size Classification
    • MCV
    • Microcytic- <80fL
    • Normocytic- 80-100fL
    • Macrocytic- >100fL
  69. Target Cell
    • Seen in
    • Hemoglobin C disease
    • Liver Disease
    • Iron Deficiency
    • Hgb abnormalities
    • Thalassemia
    • or artifact
  70. Spherocytes
    • Increased MCHC, no central pallor
    • Loss of RBC membrane
    • Ab and complement coated cells get their membranes shreard in splenic circulation
    • Also hereditary
  71. Stomatocytes
    • Slit like central pallor
    • Increased fragility
  72. Ovalocytes and Elliptocytes
    • ATP and 2,3-DPG decreased
    • Ovalocytes-myelodysplastic syndromes, thalassemias, megaloblastic processes
    • Eliptocytes-seen in hereditary elliptocytosis, ideopathic myelofibrosis and iron deficiency
  73. Image Upload
    Ovalocytes and Elliptocytes
  74. Acnthocytes
    • Seen in:
    • abetalipoproteinemia (a lipid disorder)
    • anorexia nervosa
    • lecithin cholestrol acyltransferase deficiency
    • alcohol intoxication
    • pyruvate kinase deficiency
    • vit E deficiency
    • post splenectomy
    • MCHC normal
  75. Image Upload
    Acanthocytes
  76. Tear Drops
    • RBC inclusion is “pitted” as cell passes through the spleen. A “tail” is left and RBC membrane does not reform discocyte shape.
    • Large cells have to squeeze through microvasculature of the spleen
    • Seen in ideopathic myelofibrosis with myeloid metaplasia, thalassemia syndromes, iron deficiency, inclusion bodies (the things that get “pitted” see later in lecture)
  77. Image Upload
    Tear Drop Cells
  78. Fragmented Cells
    • Helmet Cells
    • Burr Cells
    • Shistocytes
  79. Red Cell Inclusions
    • •Howell Jolly Bodies–remnants of DNA
    • •Siderotic Granules/ Pappenheimer bodies–iron
    • •Basophilic stippling–RNA and mitochondrial remnants
    • •Heinz Bodies–denatured hemoglobin
  80. Heinz Bodies
    • Denatured and precipitated Hgb
    • Use crystal violet or brilliant cresyl blue stain.
    • α thalassemic syndromes, G6PD, unstable Hgb syndromes, RBC injury from chemical insult
  81. Cabot Rings
    • figure 8 shaped stippling
    • Made of arginine-rich histones and non-hemoblobin iron
    • Seen in megaloblastic anemias, homozygous thalassemic syndromes, post splenectomy
  82. Image Upload
    Cabot Ring
  83. Siderotic Granules (Prussian Blue)
    and
    Pappenheimer Bodies (Wright Stain)
    • •Granules of non-heme iron
    • •Seen in iron excess, sideroblastic anemia, hemochromatosis, hemosiderosis, sickle cell, thalassemia, post splenectomy
  84. Image Upload
    Pappenheimer bodies
  85. Rouleaux Formation
    • Multiple myeloma
    • Waldenstrom’s macroglobulinemia
    • Chronic inflammatory disorders
    • Some lymphomas
  86. Microcytic Anemias
    • Iron deficiency anemia
    • Sideroblastic anemia
    • Thalassemia
    • Anemia of inflammation
  87. Iron Deficiency Anemia
    • Decreased red count
    • Decreased hemoglobin
    • Decreased hematocrit
    • Decreased MCV
    • Decreased MCHC
    • Decreased serum iron
    • Decreased serum ferritin
    • Increased TIBC
    • Decreased transferrin sat.
  88. Anemia of Chronic Disease/Anemia Inflammation (AOI)
    • Red cell count, hemoglobin, and hematocrit all borderline
    • MCHC normal
    • MCV slightly low
    • Serum iron low
    • Serum ferritin normal or increased
    • TIBC decreased-holding onto iron
  89. Sideroblastic Anemia
    • 5-aminolevulinic synthetase is deficient, Protoporphyrin ring cannot be formed
    • Iron accumulates in the mitochondria, because it cannot be incorporated into heme
    • Dimorphic blood picture
    • Pappenheimer bodies
    • Siderocytes and ringed sideroblasts with Prussian blue (BM)
    • Increased serum ferritin
    • Increased serum iron
    • TIBC is normal or low
  90. Iron Overload and Hemochromatosis
    • (NOT AN ANEMIA!)
    • accumulation of excess iron in reticuloendothelial cells in various
    • tissues
    • Causes tissue damage
    • Increase in intestinal iron absorption due to mutation in the HFE gene on chromosome 6
  91. Hereditary Hemochromatosis
    • increased % transferrin saturation is a hallmark
    • presence of HFE gene
    • Elevated serum iron
    • Elevated serum ferritin
    • Elevated transferrin saturation
    • Normal serum transferrin
    • Normal TIBC
  92. Thalassemia
    • Globin chain disorders
    • Microcytic process
    • Hemolytic anemias
    • Normal iron status
    • Not enough Hgb A chains produced
  93. Alpha Thal
    • 0, 1 or 2 functional genes.
    • Also a carrier state with 3 functional genes
    • Gene Deletions
  94. Beta Thal
    • Inherit defective beta genes
    • Heterozygotes or homozygotes
    • Many mutations
  95. Image Upload
    H-Bodies w. supravital stain
  96. Osmotic Fragility
    • Used to screen for carrier states of thalassemia
    • (and also hereditary spherocytosis)
    • Normal RBC’s lyse at .45% NaCl
    • HE spherocytes (decreased SA:volume) lyse at .65% NaCl
    • Beta thal and alpha thal trait lyse at < .45% NaCl
  97. Beta thalassemia major
    Cooley’s Anemia
    • Little or no beta-chains (therefore little or nohemoglobin A)
    • Low hemoglobin levels (6 - 9 g/dl)
  98. Beta thalassemia minor/trait
    • 1 abnormal beta gene
    • Microcytic, hypochromic anemia
    • Hemoglobin A2 increased to 5-10% Alpha/Delta combos
  99. Kleihauer-Betke acid elution test
    • Hb F
    • Normal cells = ghost
    • HGB F cells-stained
  100. Macrocytic Anemias
    • Macrocytic Megaloblastic Anemias
    • –Vit B 12 or Folic Acid Deficiencies
    • Macrocytic Non-Megaloblastic Anemias
    • –Liver Dz. or drug induced
  101. Megaloblastic Anemia
    • Macrocytic normochromic anemia
    • Defective nuclear maturation due to impaired DNA synthesis
    • N:C asynchrony
    • Cause -Vit B 12 or Folic acid deficiency
    • MCH is increased, but MCHC is normal
  102. Megaloblasts
    • Megaloblasts = large RBC precursors
    • Large granulocyte precursors including large bands and giant metamyelocytes
    • “sievelike“ chromatin
  103. Megaloblastic Smear
    • Macrocytes and macro-ovalocytes
    • Schistocytes, tear drops, spherocytes, targets
    • HJ bodies, Cabot rings, basophilic stippling
    • Absolute retic is low, RPI is <2 indicating ineffective erythropoeisis
  104. Reticulocyte
    Corrected retic% = (retic% X Patient hct)/Normal hct* based on age and sex
  105. RPI
    • Reticulocyte Production Index
    • RPI = corrected retic / maturation time in days

Card Set Information

Author:
mawilburn
ID:
12654
Filename:
Hematology Exam 2
Updated:
2010-05-13 18:38:59
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Blood Heme Melissa
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Exam 2 heme
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