HEME_MS2

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HEME_MS2
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2011-09-17 00:32:23
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HEMATOLOGY MS2
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HEMATOLOGY MS2
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  1. MACROCYTIC w RETIC INDEX > 2
    HEMORRHAGE

    HEMOLYSIS
  2. MACROCYTIC w RETIC INDEX < 2
    • MEGALOBLASTIC
    • --B12 DEF
    • --FOLATE DEF
    • --DRUG INDUCED
    • --MDS

    • NONMEGALOBLASTIC
    • --ALC
    • --LIVER DISEASE
    • --APLASTIC ANEMIA
    • --ENDOCRINOPATHY
  3. NORMOCYTIC w RETIC INDEX > 2
    HEMORRHAGE

    HEMOLYSIS
  4. NORMOCYTIC w RETIC INDEX < 2
    • STEM CELL
    • --APLASTIC ANEMIA
    • --RBS APLASIA

    LIVER DISEASE

    RENAL DISEASE

    CHRONIC DISEASE
  5. MICROCYTIC w LOW SERUM Fe
    Fe DEF

    CHRONIC DISEASE
  6. MICROCYTIC w nL OR HIGH SERUM Fe
    THALASSEMIA

    Hg-OPATHY

    SIDERBLASTIC ANEMIA

    LEAD INTOX

    PORPHYRIA
  7. DEFINITION OF MICROCYTIC ANEMIA
    ANEMIA PRESENTING WITH MCV < 80
  8. Fe DEFICIENCY -- CLINICAL FINDINGS
    MICROCYTIC ANEMIA

    KOILONYCHIA -- spoon shaped nails

    PICA

    ESOPHAGEAL WEBS -- PLUMMER-VINSON SYND

    CHLOROSIS -- GREEN-TINGED SKIN COLOR
  9. CBC LAB FOR Fe DEFICIENCY
    • LOW:
    • --Hgb/Hct/RBC
    • --MCV
    • --MCHC

    • HIGH:
    • --RDW
    • --Plt Ct
  10. MORPHOLOGY OF Fe DEFICIENCY
    ANISOCYTOSIS

    POIKILOCYTOSIS

    HYPOCHROMIA

    PENCIL-SHAPED CELLS

    TARGET CELLS

    ELLIPTOCYTES TYPICAL OF ANEMIA BUT NOT Dx

    (Fe DEF COVERED IN MICROCYTIC ANEMIA LECT)

  11. IRON METABOLISM
    TOTAL BODY IRON ~ 2-3,000 mg

    TOTAL STORAGE IRON ~ 1,000 mg

    • DIET
    • --ABS 10% OF INTAKE ~ 1mg

    --TRANSPORT - TRANSFERIN, CAPACITY TO BIND ~ 300ugFe/dL

    --USAGE - MOSTLY BY RBCs TO MAKE Hg, SMALL AMOUNTS USED TO PROD NON-HEME ENZs. 1ml PACKAGED RBC (2ml whole blood) IS ~ 1mg/day

    --STORAGE - FERRITIN = APOFERRITIN + IRON; STORAGE IN BN MW OTHER SITES. INC FERRITIN ~ INFLAM

    --EXCRETION - 1mg/day
  12. CAUSES OF Fe DEFICIENCY
    DIET -- #1 WORLDWIDE; USA INFANTS AND CHILDREN

    BLOOD LOSS -- MENSTRAL, OCCULT, GI

    DEC ABS
  13. SERUM IRON STUDIES
    •Serum Fe

    • •TIBC – Total Iron Binding Capacity of
    • Transferrin

    •% Saturation = Fe/TIBC

    • •Ferritin – serum level closely follows
    • storage amount
  14. CHART OF MICROCYTIC ANEMIAS
  15. Fe LABS FOR Fe DEFICIENCY
    SERUM Fe (--)

    TIBC ++

    %SAT <10 (--)

    FERRITIN (-)

  16. Fe LABS FOR CHRONIC DISEASE
    L SERUM Fe

    TIBC (nL or -)

    %SAT <15 (-)

    FERRITIN (nL OR +)

    MICROCYTIC ANEMIA
  17. Fe LABS FOR THALASSEMIA
    ALL nL

    MICROCYTIC ANEMIA
  18. Fe LABS FOR SIDEROBLASTIC ANEMIA
    MICROCYTIC ANEMIA

    SERUM Fe ++

    TIBC (nL OR -)

    %SAT ++

    FERRITIN ++
  19. EVOLUTION OF IRON DEFICIENCY
    MICROCYTIC ANEMIA

    BN MW STORAGE OF IRON IS DEPLETED

    SERUM FERRITIN DEC THEN SERUM Fe DEC AND TIBC INC

    RBC PRECURSORS DEC IN SIZE & RDW INC. NO MCV DEC YET

    Hgb/Hct BEGIN TO DEC

    MCV DEC

    RBC BECOME HYPOCHROMIC

    MCHC DEC
  20. PATHOGENESIS OF ANEMIA IN CHRONIC DISEASE
    MICROCYTIC ANEMIA

    CAUSES DEC PROD OF RBC AS WELL AS AN IMPAIRED IRON UTILIZATION

    STORAGE IRON IN MACROs OF THE BONE MARROW IS NOT RELEASED TO THE RBC PRECURSORS. THEREFORE, FERRITIN IS INC, SERUM Fe IS DEC, AND TIBC IS nL OR DEC

    --CHRON MICROBIAL INF SUCH AS OSTEOMYELITIS, TB, ABCESS, ENDOCARDITIS

    --IMMUNE DISORDERS SUCH AS RHEUM ARTH

    --CHRON GI DISORDERS

    --NEOPLASMS - VARIOUS CARCINOMAS, LYMPHOMAS
  21. SIDEROBLASTIC ANEMIA DEFINITION AND CLASSIFICATION
    MICROCYTIC ANEMIA

    • DEFINED BY BONE MARROW FINDINGS
    • --15% RBC PRECURSORS MUST BE RINGED SIDEROBLASTS (rbc precursor w iron abnormally in mito that rim the nuclear mem giving an appearance of blue pearl necklace)

    • HEREDITARY
    • --X LINKED
    • --AUTOSOMAL RECESSIVE
    • --d-AMINOLEVULINIC ACID SYNTHETASE (ALA)

    • ACQUIRED
    • --DRUG INDUCED
    • --TOXIN
    • --DISEASE ASSOC
    • --IDIOPATHIC (myelodysplastic state)

  22. LEAD TOXICITY
    MICROCYTIC ANEMIA

    HYPOCHROMIC

    ABL BASOPHILIC STIPPLING

    RBC CHANGE LATE IN DISEASE COURSE

    LEAD INH HEME PROD AT SEVERAL POINTS, AND SPECIFICALLY, INH ALA DEHYDRATASE AND FERROKETOLASE
  23. MACROCYTIC ANEMIA DEFINITION
    MCV >= 100
  24. MEGALOBLASTIC ANEMIA DEFINITION
    Grp OF ANEMIAS w DEFECTIVE DNA SYNTH

    RESULTING IN ABN LARGE ERYTH PRECURSORS (megaloblasts)

    • MOST OFTEN CAUSED BY VIT B12 OR FOLATE DEF
    • --BOTH NEC IN PROD OF THYMIDINE FOR DNA SYNTH
    • --DOES NOT EFFECT RNA SYNTH FOR TRANSCRIPT/TRANSLATE OF GENES

    NUC REMAIN IMMATURE WHILE CELL GROWS AND CYTO MATURES

    HEMATO CELLS MOST APPARENT, BUT AFFECTS ALL OTHER CELLS UNDERGOING MITOTIC DIVISION

    •Defective DNA synthesis due to decreased thymidine production

    •Most cases are due to B12/Folate deficiency

    • •RNA uses uracil, not thymidine, hence transcription/translation is not
    • affected

    • •In developing cells, nuclei remain large and immature, cytoplasm continues to
    • mature
  25. CLINICAL FINDINGS OF MEGALOBLASTIC ANEMIA AND CBC LAB VALUES
    GLOSSITIS AND VAGUE ABN PAINS

    INH OF DNA SYNTH --> CAN'T REPLACE GI LINING

    –Hgb/Hct/RBC ↓

    MCV ↑

    MCHC Nl

    –Plt Ct nl or ↓

    WBC nl or ↓

    –LDH ↑↑

    T. Bili nl or ↑

    Indirect Bili ↑
  26. PIC MICROCYTIC BONE MARROW IRON STAIN
  27. RBC MORPHOLOGY OF MEGALOBLASTIC ANEMIA
    PREDOMINANTLY MACRO-OVALOCYTES

    ANISOCYTOSIS AND POIKILOCYTOSIS

    ALSO FEW FRAG RBCs

    HYPERSEG NEUTS (>5 lobes)

    HYPERCELLULAR BONE MARROW

  28. B12 METABOLISM AND ABSORPTION
    MACROCYTIC ANEMIA

    •Daily requirements – 1-2 µg

    •Body stores – 3,000 – 5,000 mg

    •If diet lacks B12, 5-6 years for deficiency to develop

    •Dietary source – animal origin foods

    • B12 ABS REQ IF
    • --ONLY ABS IN TERM ILEUM
    • --BINDS TO TRANSCOBALAMIN FOR TRANS


    B12 = COBALAMIN
  29. CAUSES OF B12 DEFICIENCY
    •Pernicious anemia

    •Dietary lack

    •Diphyllobothrium latum

    • •Bacterial overgrowth (Blind-loop
    • syndrome)

    •Malabsorption syndrome

    •Transcobalamin deficiency

    •Drug inhibition
  30. PERNICIOUS ANEMIA
    B12 DEFICIENCY

    •Atrophic gastritis, hypochlorhydria, decreased or absent IF

    •Anti-parietal cell antibodies sensitive (present in 90%), but not specific

    •Anti-Intrinsic factor antibodies specific (75%), but not sensitive

    • •Schilling test
    • --LOADING DOSE OF IM B12
    • --ORAL LABELED B12 --> OUTPUT MEASURED IN URINE
    • --IF LOW --> GIVE w I.F. --> RETURN TO nL THEN IF DEFICIENCY
    • --IF STILL LOW, THEN MALABSORPTION OTHER THAN I.F.
  31. ADDITIONAL FEATURES OF PERNICIOUS ANEMIA OTHER THAN IF DEFICIENCY
    • MYELIN MAINTENANCE
    • --B12 DEFICIENCY --> DEMYELINATION OF POSTERIOR AND LAT COLUMNS OF SPINAL CORD
    • --CAN OCCUR w/o ANEMIA

    • LOW RBC FOLATE
    • --B12 AIDS IN PASSING FOLATE THROUGH RBC MEM (most consistent measure of folate

    LARGE DOSES OF FOLATE CAN CORRECT ANEMIA IN B12 DEF, BUT NEURO DAMAGE WILL CONTINUE AND BECOME IRREV
  32. FOLATE METABOLISM AND Dx
    • Dx
    • --SERUM AND RBC FOLATE LEVELS
    • --SERUM FOLATE FLUCs QUICKLY, HENCE RBC FOLATE IS MORE ACCURATE

    •Daily requirements – 200 µg

    •Body stores – 20 - 70 mg

    •If diet lacks Folate, 3 months for deficiency to develop & MEGALOBLASTIC ANEMIA

    •Dietary source – vegetables (esp. green leafy) and fruits (heat labile)

    •FOLATE (Pterylmonoglutamic acid) is in food as polygutamates

    •After ingestion, split into monoglutamates

    •Absorbed in proximal jejunum

    •Converted to 5-methyltetrahydrofolate for transport
  33. MEGALOBLASTIC ANEMIA Dx
    •Serum B12 level

    •Serum Folate level (fluctuates)

    •RBC Folate level better indicator of folate stores

    •Large doses of folate can improve anemia in B12 deficiency, but not the neurologic affects

    • OTHER CAUSES OF MEGALOBLASTIC ANEMIA
    • --DRUG/TOXIN
    • --MYELODYSPLASTIC STATES
  34. MEGALOBLASTIC ANEMIA Tx
    FOLATE and/or B12 DEFICIENCY

    •Once treated with appropriate substance:

    –Megaloblastic changes in BM revert to normal maturation within hours

    –Reticulocytes begin to increase in 2-3 days

    –10 days – two weeks for peak retic rise

    –4 weeks for Hgb to improve

    –Variable time for Hgb to return to normal

    • –Large doses of Folate can correct anemia in B12 deficiency, but neurologic deficits will progress and
    • become irreversible
  35. SUMMERY OF MEGALOBLASTIC ANEMIA FINDINGS
    • •Macrocytic anemia, macro-ovalocytes
    • --NRBC and Howell-Jolly bodies

    •Pancytopenia, hypersegmented neuts

    •Hypercellular bone marrow

    •Increased LDH

    •Increased Total bilirubin d/t indirect bili
  36. APLASTIC ANEMIA AND LAB VALUES AND PIC
    • NON-MEGALOBLASTIC MACROCYTIC ANEMIA
    • --MCV >= 100 w ROUND, NOT OVAL MACROCYTES

    • •Failure of hematopoietic stem cell resulting in pancytopenia
    • --MARKEDLY HYPOCELLULAR BONE MARROW

    • •Clinical Findings:
    • –Usual signs/symptoms of anemia

    –Petechiae

    –Bacterial infections

    •Laboratory Findings:

    • Hgb/HctRBC ↓
    • WBC ↓
    • Plt Ct ↓
    • MCV nl. or ↑

    • nL

    • APLASTIC
  37. CAUSES OF APLASTIC ANEMIA
    •Inherited – Fanconi anemia

    •Acquired

    –Idiopathic (most common)

    –Chemical agents

    –Idiosyncratic drug reaction

    –Radiation

    –Viral infections

    Miscellaneous
  38. OTHER CAUSES OF NON-MEGALOBLASTIC MACROCYTIC ANEMIA
    ALC ABUSE

    LIVER Dz -- MORE TARGET CELLS

    ENDOCRINOPATHIES
  39. HEMOLYTIC ANEMIA DEFINITION AND CATEGORIES
    •Hemolysis - RBC have a shortened live span, < 120 days

    •RBC are destroyed either within the peripheral blood circulation = intravascular

    •Or, RBC are destroyed outside of the peripheral blood - extravascular
  40. DEFINITION OF INTRAVASCULAR HEMOLYSIS
    RBCs FRAGMENTED IN BLOOD STREAM

    RELEASED Hg BIND TO HAPTOGLOBIN --> REMOVED BY LIVER WHERE Hg BROKEN DOWN

    WHEN HAPTOGLOBIN DEPLETED, FREE Hg CLEARED THROUGH RENAL TUBULES AND APPEARS IN URINE

    SOME ABS BY TUBULAR CELLS, BROKEN DOWN, AND IN FEW DAYS CONTAIN HEMOSIDERIN SEEN IN URINE SEDIMENT (hemosiderinuria)
  41. INTRAVASCULAR HEMOLYSIS LAB VALUES
    SERUM HAPTOGLOBIN -

    PLASMA Hg +

    URINE Hg +

    URINE HEMOSIDERIN + (after several days)

    LDH +

    PERIPHERAL BLOOD SMEAR - SCHISTOCYTES
  42. EXTRAVASCULAR HEMOLYSIS DEFINITION AND LAB VALUES
    RBCs DESTROYED OUTSIDE OF BLOOD STREAM BY MACROPHAGES

    USUALLY IN THE SPLEEN

    REMNANTS OF RBCs FORM MICRO-SPHEROCTES

    MACROPHAGES PROCESS Hg & RELEASE END PROD BILIRUBIN

    --> BILI BINDS TO ALBUMIN AND CLEARED BY LIVER

    INC BILI INTO GI CONVERTED TO UROBILINOGEN WHICH IS REABSORBED AND EXCRETED IN URINE

    INDIRECT BILI +

    URINE UROBILI +

    LDH +

    • PERIPH BLOOD SMEAR =
    • MICRO-SPHEROCYTES
  43. CAUSES OF INTRINSIC HEMPLYTIC ANEMIA
    Inherent defect of RBC, most often inherited, involving membrane, hemoglobin or enzymes

    • •Membrane Disorders
    • –Spherocytosis
    • –Elliptocytosis
    • –Stomatocytosis

    • •Hemoglobinopathy
    • –SS Disease
    • –Thalassemias
    • –Others

    • •Enzyme Deficiency
    • –G-6-PD Deficiency
    • –Pyruvate kinase deficiency
    • –Paroxysmal Hgb
  44. CAUSES OF EXTRINSIC HEMOLYTIC ANEMIA
    RBC is inherently normal, something extrinsic to RBC is causing damage, most often immune-related, vascular, infectious, toxic or mechanical
  45. HEREDITARY SPHEROCYTOSIS
    INTRINSIC HEMOLYTIC ANEMIA

    MEM DEFECT

    •Autosomal dominant

    •Ankyrin/spectrin gene mutations

    •Spherical shaped RBC are sequestered/destroy by spleen

    •Variable clinical course

    SPLENECTOMY MAY BE BENEFICIAL TO REDUCE HEMOLYSIS, BUT RBCs STILL SPHERICAL

    OSMOTIC FRAGILITY TEST
  46. nL AND ABnL GLOBIN CHAINS
    Major Normal Hemoglobins:

    A α2β2 (adult)

    A2 α2δ2

    F α2γ2 (fetal)

    Abnormal Hemoglobins:

    Bart’s γ4

    H β4

    S α2β2 6glutamic→valine

    C α2β2 6glutamic→lysine
  47. OVALOCYTE/ELLIPTOCYTE
    ELONGATION OF RBC WITH OVAL OR ELLIPTICAL SHAPE

    NON-SPECIFIC FINDING

    CAN BE INC w CHEMOTHERAPY

    MANY ELLIPOs INDICATE HEREDITARY ELLIPTOCYTOSIS (autosomal dominant)
  48. SPHEROCYTE
    LOSS OF MEM SURFACE AREA CAUSING SPHERE SHAPE

    APPEAR ROUND w DENSE STAINING AND NO CENTRAL PALLOR

    MICROSPHEROCYTES ARE INDICATIVE OF EXTRAVASCULAR HEMOLYSIS

    nL SIZED SPHEROCYTES INDICATIVE OF HEREDITARY SPHEROCYTOSIS (autosomal dominant)

  49. STOMATOCYTE
    CENTRAL PALLOR IS SLIT-LIKE INSTEAD OF ROUND

    NON-SPECIFIC FINDING -- POSS IN MYELODYSPLASTIC STATES

    ASSOC w Rh NULL Dz, HEREDITARY STOMATOCYTOSIS (autosomal dominant)

  50. TARGET CELL
    CODOCYTE

    CAUSED BY INC CELL MEM FOR AMOUNT OF Hg

    ASSOC w LIVER Dz, ASPLENIA, HYPOCHROMIC ANEMIA, AND Hg-opathies
  51. TEAR DROP CELL
    DACROCYTE

    NON-SPECIFIC FINDING

    ASSOC w MYELOFIBROSIS

  52. SCHISTOCYTE
    FRAG RBCs

    INDICATIVE OF INTRAVASC HEMOLYSIS

    CAN BE HELMET OR BITE SHAPED CELL

  53. ACANTHOCYTE
    SPUR CELL

    MEM HAS LONG PROJs, NONSYM DISTRIBUTION, w BULBOUS ENDS

    ASSOC w SOME TYPES OF LIVER Dz AND a-b-LIPOPROTEINEMIA

  54. BURR CELL
    ECHINOCYTE

    MEM HAS SHORT SPIKES, SYM DISTRIBUTION, SHARP POINTED ENDS

    ASSOC w RENAL FAILURE

  55. CRENATED CELL
    PERIPH MEM SHAP IS SCALLOPED

    USUALLY DRYING ARTIFACT BUT CAN BE ASSOC w HYPEROSMOLALITY

  56. SICKLE CELL MORPHOLOGY
    DEPRANOCYTE

    CELL IS ELONGATED AND CURVED w POINTED ENDS

    MOST OFTEN SEEN IN S-S Dz

    CAN BE ASSOC w FEW OTHER Hg-OPATHIES
  57. BASOPHILIC STIPPLING
    RBC INCLUSIONS

    SMALL BLUE DOTS DISTRIBUTED THROUGHOUT THE CELL

    REMNANTS OF RNA

    FINE STIPPLING IS INDICATIVE OF YOUNF RBC

    • COURSE STIPPLING IS SEEN IN: --THALASSEMIA
    • --LEAD INTOX
    • --MYELOBLSTIC STATES

  58. HOWELL-JOLLY BODY
    RBC INCLUSION

    USUALLY SINGLE, DENSE ROUND INCLUSION OF MAGENTA COLOR

    REMNANT OF NUCLEUS

    MOST OFTEN SEEN IN ASPLENIA, BUT ALSO IN MEGALOBLASTIC ANEMIA AND MYELODYSPLASTIC STATES
  59. NUCLEATED RBC
    NUC RBC PRECURSORS SHOULD NOT BE SEEN IN PERIPH CIRC

    IF PRESENT, MAY BE ASSOC w STRESS SUCH AS ACUTE HEMORR, SEVERE ANEMIA, HEMOLYSIS, etc, OR TRUE NEOPLASM
  60. PAPPENHEIMER BODY
    RBC INCLUSION

    SMALL BLUE INCLUSIONS

    USUALLY ECCENTRICALLY LOCATED, ONE OR SEVERAL

    CONSISTS OF STAINABLE IRON

    ASSOC w IRON OVERLOAD IN MARROW AND ASPENIA

    CELL w PAPP BODIES IS A SIDEROCYTE,

    NUCLEATED RBC w IRON IS A SEDEROBLAST,

    NUCLEATED RBC w IRON AROUND NUC IS A RINGED SIDEROBLAST
  61. HEINZ BODY
    RBC INCLUSION

    INLY SEEN WITH SUPRA-VITAL STAINING

    CONSISTS OF PRECIPITATED Hg

    INDICATIVE OF UNSTABLE Hg

    ASSIC w G-6-PD DEFICIENCY, SOME DRUGS, AND SOME Hg-OPATHIES

  62. POLYCHROMASIA
    DIFFUSE BLUE COLOR OF RBC

    REMNANT OF RNA

    INDICATIVE OF YOUNG RBC (RETICULOCYTE)
  63. RETOCULOCYTE
    YOUNG RBC THAT IS SLIGHTLY LARGER THAN MATURE RBC

    MAY BE SEEN AS POLYCHROMASIA, BUT BEST DETECTION IS BY RETICULOCYTE COUNT

  64. ROULEAU
    STICKING TOGETHER OF RBC

    DUE TO LOSS OF ZETA POTENTIAL CAUSED BY INC FIBRINOGEN OR GAMMA GLOBULINS

    RBCs NORMALLY (-) CHARGE
  65. COLD AGGLUTININS
    AGGLUTINATED CLUMPS OF RBC DUE TO AUTO ANTI-BODIES OF IgM
  66. RETICULOCYTE INDEX
    RC X (Pt Hct / 45) x 1/F = RI


    1 45

    1.5 35

    2 25

    2.5 15
  67. ANEMIA -- GENERAL CLINICAL FINDINGS
    •Weakness, malaise, fatigue

    •Dyspnea on exertion

    •Pallor – skin, conjunctiva, nail beds

    •Angina

    •Headache, faintness, dim vision
  68. RBC PARAMETERS
    •RBC count

    •Hematocrit

    •Hemoglobin

    •MCV - Mean cell volume

    •MCH – Mean cell hemoglobin

    •MCHC – Mean cell hemoglobin conc.

    •RDW – Red cell distribution width
  69. BETA-THALASSEMIA
    HEMOLYTIC ANEMIA

    •Β-Thal Major

    • –Severe
    • anemia, 6-9 months

    –Hemolysis

    –↓↓ or absent HbA

    • –Skeletal
    • changes

    • –Requires
    • transfusions

    • –Secondary
    • hemochromatosis

    • –Β0/β0, β+/β+
    • -----------------------------------

    •β-Thal Intermedia

    –β0/β and β+/β+

    • –Moderate microcytic anemia not requiring transfusion
    • ----------------------------------------

    •β-Thal Minor

    –β0/β and β+/β

    • –Microcytosis
    • with or without anemia

    –MCV < 80, RBC may be normal count

    –RDW normal

    –Hb A2 is mildly increased, 4 – 8%
  70. ALPHA-THALASSEMIA
    •Alpha chain gene locus chromosome 16

    •Two loci, hence 4 alleles

    •Most common cause of alpha-thalassemia is gene deletion as opposed to mutation

    •Normal genotype αα/αα

    •-α/αα silent carrier, no anemia

    •--/αα, -α/-α similar to beta-thal minor with microcytosis, mild anemia

    • •--/-α resembles beta-thal intermedia,
    • forms HbH

    •--/-- lethal to fetus; hydrops fetalis
  71. ENZYME DEFICIENCIES LEADING TO HEMOLYSIS
    PROBS w HEXOSE MONOPHOSPHATE SHUNT OR GLUTATHIONE MET MAY LEAVE RBC VULN TO OX INJ

    • G-6-P DEHYDROGENASE DEFICIENCY
    • --X-LINKED RECESSIVE
    • --MANY VARIANTS BUT ONLT 2 HARMFUL

    • OXIDANT STRESS
    • --GLOBIN CHANS BECOME UNSTABLE
    • --PRECIPITATE AS HEINZ BODIES
    • --INTRA/EXTRA VASCULAR HEMOLYSIS

    SELF LIMITED AS OLDER RBCs ARE MOST VULNERABLE

    ANTI-MALARIALS, SULFONAMIDES, VARIOUS INF, INGESTION OF FAVA BEANS
  72. PARAXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH)
    RARE

    • ACQUIRED INTRINSIC RBC DEFECT
    • --MUTATION IN PHOPHATIDYLINOSITOL GLYCAN A
    • --PROD CERTAIN MEM PROTS

    RBC SENSITIVE TO LYSIS BY COMPLEMENT DUE TO DEFICIENCY OF CERTAIN CELL MEM PROTS

    PLATELETS AND WBCs MAY BE SIMILARLY AFFECTED
  73. SICKLE CELL GENETICS
    • •Sickle Hb
    • – Beta chain gene point mutation causes one AA substitution:

    6th position, valine substituted for glutamic acid

    •HbS, with de-oxygenation, polymerizes

    •Polymerized HbS may be reversible to a point

    •RBC becomes deformed into “sickle” shape

    •Heterozygote genotype: β/β6glutamic→valine

    • Sickle trait:
    • 40% HbS
    • 60% HbA

    •Usually asymptomatic

    • •Can have sickle crisis if under extreme conditions
    • --------------------------------------------------

    •Homozygous genotype:

    β6glutamic→valine/β6glutamic→valine

    •HbF is protective until 6-9 months of age

    •Patient’s have a hemolytic anemia

    •Most serious problems relate to irreversible sickling and vaso-occlusion
  74. SICKLE CELL COMPLICATIONS
    Autosplenectomy

    Vaso-occlusive crisis

    • Sequestration crisis
    • --CHILDREN w INTACT SPLEENS
    • --MASSIVE ENTRAPMENT OF SICKLE RBC --RAPID SPLENIC ENLARGEMENT, HYPOVOLEMIA, SHOCK

    Infections with encapsulated organisms

    • Aplastic crisis
    • --RBC PROGENITOR CELLS INF w PARVOVIRUS B19
    • -- AFTER MARROW GIVES OUT, CAN REGENERATE
  75. Hg-C
    2ND MOST COMMON STRUCTURALLY ABN Hg DETECTED IN U.S.

    MAY HAVE MILD HEMOLYSIS

    MANY TARGET CELLS
  76. IMMUNE RELATED CAUSES OF OF HEMOLYTIC ANEMIA
    EXTRINSIC DEFECTS OF RBC

    Auto-immune

    •“Warm antibody” IgG

    • •Diagnostic test is DAT
    • --DIRECT ANTI-GLOBULIN TEST

    •Can be acute or chronic

    •Usually associated with other autoimmune disorders (SLE)

    •Most hemolysis is extra-vascular

    COLD ANTIBODY DUE TO IgM AUTO-ANTIBODIES (rarely in vivo)

    • Allo-immune
    • --FROM TRANSFUSION

    • Drug induced
    • --Dg STICKS TO RBC AND Ab BINDS TO Dg
    • --ANTI-Dg Ab BINDS TO Dg, AND IMMUNE COMPLEX ACTIVATES COMPLEMENT
    • --Dg INDUCES FORMATION OF AUTO-Ab TO INTRINSIC Ag ON RBC MEM
  77. MICRO-ANGIOPATHIC CAUSES OF HEMOLYTIC ANEMIA
    EXTRINSIC DEFECTS OF RBC

    BUT CAUSES INTRAVASCULAR HEMOLYSIS

    RBCs CHEWED UP IN BLOOD STREAM BY SMALL VESSLES

    RBCs MECHANICALLY SHEARED AS PASS THROUGH

    • –Thrombotic thrombocytopenic purpura
    • ----MUST Dx QUICKLY
    • ----FRAG RBCs FOUND

    –Disseminated intravascular coagulation

    –Hemolytic uremic syndrome

    –Severe vasculitis
  78. EXTRINSIC DEFECTS AFFECTING RBCs
    •Immune Related

    •Micro-angiopathic

    • •Infectious agents
    • --Direct hemolysis by micro-organism
    • --Toxin production

    •Chemical agents

    • •Mechanical
    • --March hemoglobinuria
    • --Prosthetic Heart valve
  79. Thrombotic Thrombocytopenic Purpura (TTP)
    MICROANGIOPATHIC HEMOLYTIC ANEMIA

    PLATELET AND FIBRIN THROMBI FORM IN SMALL ARTERIOLES AND CAPILLARIES IN WIDESPREAD DISTRIBUTION

    • MUST Dx QUICKLY
    • --MUST DISTINGUISH FROM DIC
    • --DIC HAS FIBRIN THROMBI AND INC PT AND aPTT
    • --TTP HAS PLATELET THROMBI AND nL PT AND aPTT

    •Deficiency of vWF cleaving-protease

    •Most cases are acquired

    •F:M 3:2

    •Most cases are fulminant – survival directly relates to time period of initiation of therapy

    • PENTAD OF CLINICAL SYND
    • --THROMBOCYTOPENIA
    • --HEM ANEMIA
    • --NEURO ABN
    • --FEVER
    • --RENAL DYSFUNCTION

  80. PFA-100 TEST
    PLATELET FUNCTION ANALYSIS

    CEPI = COLLAGEN AND EPINEPHRINE

    CADP = COLLAGEN AND ADP

    • CEPI <180 = NORMAL PLATELET FUNCTION
    • --NO SIGNIFICANT PLATELET DEFECT

    CEPI > 180 and CADP <116, ASPIRIN OR OTHER MED EFFECT

    • CEPI > 180 and CADP >116, ABN PLATELET FUNCTION
    • --THROMBOCYTOPENIA AND ANEMIA SHOULD FIRST BE EXCLUDED
  81. PROTHROMBIN TIME
    PT -- EXTRINSIC CLOTTING SYSTEM

    PROLONGED IN:

    • INHERITED
    • --I, II, V, VII, X

    • ACQUIRED MULTI DEFICIENCIES OF
    • --II, VII, X IN WARFARIN-TYPE ANTOCOAG THERA

    INSENSITIVE TO HEPARIN
  82. PARTIAL THROMBOPLASTIN TIME
    METHOD FOR MONITORING HEPARIN THERAPY AND CLOTTING DEFICIENCIES

    PROLONGED TIME CAUSED 6 POSS CONDITIONS

    1) HEPARIN -- MOST COMMON CAUSE

    2) FACTOR DEF -- VIII & IX MOST COMMON. MULTI DEFs SEEN IN LIVER Dz OR DIC

    • 3) FACTOR DEF w LITTLE/NO CLINICAL SIG
    • --DEF IN "CONTACT FACTORS". MARKED PROLONGATION OF aPTT BUT NOT CAUSE BLEEDING. SEVERE DEF MAY CAUSE HYPERCOAG STATE

    4) SPECIFIC FACTOR INHIBITOR CAUSING BLEEDING -- NEARLY ALWAYS DUE TO Ab OR FACTOR VIII AND LEAD TO SEVERE BLEEDING DIATHESIS

    5) LUPUS ANTICOAG, RISK FACTOR FOR THROMBOSIS -- Ab TO PHOSPHOLIPID-PROT COMPLEXES. CAUSE PROLOGATION OF THE aPTT AND/OR OTHER COAG TESTS BUT, PARADOXICALLY, ARE ASSOC WITH HYPERCOAG and THROMBOSIS

    6) SPURIOUS
  83. MIXING STUDY OF aPTT
    IF CORRECTED ~ DEFICIENCY OF COAG FACTOR

    STILL PROLONGED ~ INHIBITOR OR COAG PRESENT
  84. THROMBIN TIME
    THROMBIN ADDED TO Pt FIBRINOGEN

    DETECT INH OF THROMBIN-FIBRINOGEN INTRACTION OR INH TO FIBRON-MONOMER POLYMERIZATION

    • PROLONGED IN PRESENCE OF:
    • --LOW CONC OF HEPARIN
    • --FIBRINOGEN DEGRADATION PRODS
    • --ABN FIBRINOGENS (fetal fibrinogen in newborn)
    • --SEVERE HYPOFIBRINOGENEMIA

    UNAFFECTED BY VIT K ANTAGONIST THERAPY AND IS NORMAL IN DEF STATES OF ALL CLOTTING FACTORS EXCEPT SEVERE FIBRINOGEN DEF
  85. QUANTITATIVE FIBRINOGEN DETERMINATION
    FIBRINOGEN < 100 ~ ONE STAGE CLOTTING TESTS WILL TEND TO BE ABN LONG DUE TO HYPOFIBRINOGENEMIA ALONE

    • ACQUIRED HYPOFIBRINOGENEMIAS CAN OCCUR SEC TO:
    • --SEVERE LIVER Dz
    • --DEPLETION PHASE OF CONSUMPTION COAG
    • --RARE COND OF PRIMARY FIBRINOLYSIS

    PRESENT POST OP, w ESTROGEN THERA, AND PREGO

    FIBRINOGEN IS ACUTE PHASE REACTANT AND RISES w INFLAM AND STRESS
  86. LIVER IN COAG
    HEPATOs SYNTH ALL NATURAL COAG INH FACTORS EXCEPT vWF

    MEGAKARYOCYTES AND ENDOTHELIUM SYNTH AND SECRETE vWF

    • VIT K
    • --FAT SOL
    • --HEPATOs, COFACTOR IN SYNTH OF FACTORS II, VII, IX, X PROT C, PROT S -- ALL BIND Ca2+ AND INTERACT w PHOSPHOLIPID

    • FIBRIN DEGRADATION PRODS CLEARED BY LIVER
    • --POST-RIBO MODIFICATION
    • --
  87. D-DIMER
    SUSPECTED VENOUS THROMBOEMBOLISM

    ELEV MODESTLY IN INTAVASCULAR (venous or arterial) THROMBOSIS

    ELEV MODESTLY WHENEVER THERE IS AN EPISODE OF HEMOSTASIS

    ELEV MILD OR MOD IN DIC (acute or chronic)

    ELEV MILD TO MOD IN PRE-ECLAMPSIA
  88. MYELOID CELL SURFACE MARKERS
    • MYELOID:
    • CD 13, 33, 11c, 34

    • PRE-B-LYMPHOBLASTIC:
    • CD 19, 20, 10, 34

    • T-LYMPHOBLASTIC:
    • CD 2, 5, 7

    • B-LYMPHOBLASTIC:
    • CD 19, 20, 10, MONOCLONAL SIg

    • SIg ~ SURFACE Ig = OLDER
    • NO SIg = YOUNGER
  89. PRE-B-LYMPHOBLASTIC CELL SURFACE MARKERS
    • MYELOID:
    • CD 13, 33, 11c, 34

    • PRE-B-LYMPHOBLASTIC:
    • CD 19, 20, 10, 34

    • T-LYMPHOBLASTIC:
    • CD 2, 5, 7

    • B-LYMPHOBLASTIC:
    • CD 19, 20, 10, MONOCLONAL SIg

    • SIg ~ SURFACE Ig = OLDER
    • NO SIg = YOUNGER
  90. T-LYMPHOBLASTIC CELL SURFACE MARKERS
    • MYELOID:
    • CD 13, 33, 11c, 34

    • PRE-B-LYMPHOBLASTIC:
    • CD 19, 20, 10, 34

    • T-LYMPHOBLASTIC:
    • CD 2, 5, 7

    • B-LYMPHOBLASTIC:
    • CD 19, 20, 10, MONOCLONAL SIg

    SIg ~ SURFACE Ig = OLDER

    NO SIg = YOUNGER
  91. B-LYMPHOBLASTIC CELL SURFACE MARKERS
    • MYELOID:
    • CD 13, 33, 11c, 34

    • PRE-B-LYMPHOBLASTIC:
    • CD 19, 20, 10, 34

    • T-LYMPHOBLASTIC:
    • CD 2, 5, 7

    • B-LYMPHOBLASTIC:
    • CD 19, 20, 10, MONOCLONAL SIg

    SIg ~ SURFACE Ig = OLDER

    NO SIg = YOUNGER
  92. ACUTE MYELOID LEUKEMIA w RECURRENT GENETIC ABNORMALITIES
    AML w t(8;21)

    FUSION OF GENES AML1 AND ETO

    5-12% OF ALL AML

    USUALLY IN YOUNGER Pts

    BM SHOWS MATURATION OF MYELOID w AT LEAST 10% PROMYELOCYTES, MYELOCYTES, AND METAMYELOCYTES. AUER RODS FREQUENT

    PROG -- USUALLY GOOD RESPONSE TO CHEMO
  93. AML w ABNORMAL BM EOSINOPHILS w
    inv(16) OR t(16:16)

    10-12% OF AMLs

    USUALLY IN YOUNGER Pts

    LEUK BLASTS SHOW EVIDENCE OF MONOCYTIC AND NEUTROPHILIC MATURATION

    BM SHOWS INC EOSINOPHILS AND PRECURSORS w ABNORMAL GRANULES.

    REARRANGEMENTS OF GENE CBFb (core binding factor beta) AT 16q22 TO MYHII (smooth musc myosin heaby chain) AT 16q13

    PROG -- RELATIVELY BETTER w HIGH RATES OF COMPLETE REMISSION AND LONG TERM REMISSION
  94. AML w t(15:17)
    ACUTE PROMYELOCYTIC LEUKEMIA

    RARa on 17, PML on 15

    5-8% OF AMLs

    PREDOM MIDDLE AGED ADULTS

    MALIG PROMYELOCYTES w VARIABLE "KIDNEY BEAN" SHAPE

    TYPICAL HYPERGRANULAR FORM w NUMEROUS PROMINENT PRIMARY GRANULES

    MULTI AUER RODS

    TRANSLOCATION -- FUSION OF RARa (RETINOIC ACID-RECEPTOR-a = vit a -- cell differentiation) w PML (proto-oncogene)

    HIGH RISK DIC DUE TO PRO-COAG ACTIVITY OF GRANULES

    GIVE VIT A (more neuts grow to maturity) FOLLOWED BY CHEMO
  95. AML w 11q23 ABNORMALITIES
    SHOWS MONOCYTIC DIFFERENTIATION w OR w/o NEUT DIFFERENTIATION

    GENE MML

    MONOCYTIC DIFF DETECTED BY +NONSPECIFIC ESTERASE & +SUDAN

    5-6% OF AMLs

    t(9;11) or t(11;19) RESULTS IN REARRANGEMENT OF GENE MLL (dev regulator) AT 11q23

    INTERMEDIATE SURVIVAL
  96. AML -- MINIMALLY DIFFERENTIATED
    NOT OTHERWISE CATEGORIZED

    >20% BLASTS IN BM

    NO AUER RODS

    NEG CYTOCHEM STAINS

    • MYELOID MARKERS BY IMMUNOPHENOTYPING
    • --CD 13, 33, 11
  97. AML -- WITHOUT MATURATION
    NOT OTHERWISE CATEGORIZED

    >20% BLASTS IN BM

    <10% CELLS OF MYELOID MATURATION

    AUER RODS MAY BE SEEN
  98. AML -- WITH MATURATION
    NOT OTHERWISE CATEGORIZED

    SIMILAR TO t(8;21)

    >20% BLASTS

    • >10% CELLS OF MATURATION
    • --PRO, META, MYELOCYTES

    FREQUENT AUER RODS SEEN

    30-45% OF AMLs

    USUALLY IN YOUNGER AGE GROUPS AND RELATIVELY BETTER PROG
  99. ACUTE MYELOMONOCYTIC LEUKEMIA (AMML)
    NOT OTHERWISE CATEGORIZED

    BLASTS SHOW MYELOID AN MONOCYTIC DIFF

    >20% but <80% MONOCYITIC CELLS

    MANY CASES INV 11q

    15-20% OF AMLs
  100. ACUTE MONOBLASTIC & MONOCYTIC LEUKEMIA
    NOT OTHERWISE CATEGORIZED

    >80% MONOCYTIC DIFF

    CLINICALLY -- MORE OFTEN HAS TISSUE INFILTRATION, esp GINGIVAL HYPERTROPHY DUE TO LEUKEMIC INFILTRATE

    MONOBLASTIC -- PREDOM OF MONOBLASTS

    MONOCYTIC -- SOME MATURATION

    11q FREQUENTLY INV

    5-8% OF AMLs
  101. ACUTE MEGAKARYOBLASTIC LEUKEMIA
    NOT OTHERWISE CATEGORIZED

    PROLIF OF MEGA-BLASTS AND ABN MATURE MEGA-CYTES

    ASSOC w FIBROSIS IN BM HAMPERING Dx
  102. DEFINITION OF MYELODYSPLASTIC SYNDROMES
    GROUP OF ACQUIRED CLONAL HEMATOPOIETIC STEM CELL DISORDERS

    CHAR CLINICALLY AND MORPH BY INEFFECTIVE HEMATOPOIESIS

    HYPERCELLULAR, DYSPOIETIC MARROW RESULTING IN INTRAMEDULLARY CELL DEATH AND PERIPHERAL CYTOPENIAS

    MACROCYTIC

    TENDENCY TO PROGRESS TO ACUTE MYELOID LEUKEMIA
  103. DEFINITION: DYSPOIESIS
    MDS

    ABNORMAL MATURATION (dysplastic) OF HEMATOPOIETIC PRECURSORS IN BM

    DETECTED MORPH AND FUNC

    ALL 3 CELL LINES OF BM INVOLVED, BUT SEVERITY OF EACH IS VARIABLE, AND INDIVIDUAL CLASSIFICATION IS REQUIRED
  104. REACTIVE vs. NEOPLASTIC LYMPHOCYTOSIS
    • REACTIVE:
    • --TRANSIENT
    • --WBC <30k
    • --HETEROGENEOUS
    • --POLYCLONAL
    • --KNOWN CAUSE

    • NEOPLASTIC:
    • --SUSTAINED
    • --WBC > 30k
    • --MONOMORPHIC
    • --CLONAL
    • --UNKNOWN IN MANY CASES
  105. B-CELL SURFACE MARKERS
    CD 19, 20, Ig kappa / lambda
  106. T-CELL SURFACE MARKERS
    CD 3, 4, 5, 7, 8

    CD 5 CAN BE PRESENT ON B-CELLS
  107. HAIRY CELL LEUKEMIA LAB OUTCOMES
    TARTRATE RESISTANT ACID PHOSPHATASE (TRAP) STAIN

    • MONOCLONAL B-CELLS
    • --STRONG SIg
    • --CD11c, CD25, CD103

    • FIBROSIS FROM BM BIOPSY -- DRY TAP
    • --FRIED EGG APPEARANCE

    SPLENOMEGALY w/o LYMPHADENOPATHY

    PANCYTOPENIA NOT LEUKOCYTOSIS
  108. CYTOGENETIC ABERRATION BCL-2
    t(14;18)(q32,q21)

    FOLLICULAR LYMPHOMA
  109. CYTOGENETIC ABERRATION C-MYC
    t(8;14)(q24;q32)

    BURKITT LYMPHOMA
  110. CYTOGENETIC ABERRATION MALT-1
    t(11;18)(q21,q21)

    MALT LYMPHOMA
  111. CYTOGENETIC ABERRATION CYCLIN D1
    t(11;14)(q13;q32)

    MANTLE CELL LYMPHOMA
  112. FOLLICULAR LYMPHOMA
    MOST COMMON INDOLENT NHL IN US (20-40%)

    50-60 yrs

    MALES = FEMALES

    • MORPHOLOGY
    • --RESEMBLES nL GERM CENTER
    • --EFFACEMENT OF NODAL ARCH
    • --NODULAR GROWTH PATTERN (may be diffuse)
    • --MIXED CENTROCYTES (small cleaved) AND CENTROBLASTS (LARGE NON-CLEAVED)
    • --LACK APAPTOSIS/TINGIBLE BODY MACROs AND ZONATION

    • IMMUNOPHENOTYPE
    • --B CELL CD 19, 20, 10
    • --MONOCLONAL (kappa or lambda LC)
    • --BCL-2 PROT

    • GENOTYPE
    • --t(14;18)(q32,q21)
    • --BCL-2 REARRANGE

    MOST Pts PRESENT IN ADVANCED STAGE (75-85% stage iv)

    MEAN SURVIVAL 7-9 yrs

    NO CURE
  113. DIFFUSE LARGE B-CELL LYMPHOMA
    MOST COMMON NHL 30-40%

    • HETEROGENEOUS GROUP OF TUMORS
    • --PREDOM LARGE LYMPHOCYTES

    30% HAVE TRANSLOCATIONS INV BCL-6 (3q27) OR MUTATION OF ITS PROMOTOR

    SOME FROM LOWER GRADE LYMPHOMAS (ex follicular)

    • SUBSET IN HIV Pts
    • --EBV + DLBCL

    • CLINICALLY
    • --RAPID GROWING MASS IN LYMPH OR EXTRANODAL (40-50%)
    • --INTERMEDIATE IN MOST
    • --HIGH GRADE ALSO

    AGGRRESSIVE BUT MAY BE CURABLE
  114. SMALL LYMPHOCYTIC LYMPHOMA
    PRIMARILY AFFECTS LYMPHOID TISSUES

    • INDISTINGUISHABLE FROM CLL EXCEPT
    • --CLL ORIGIN IN BM AND PERIPH BLD
    • --SLL ORIGIN IN LYMPHOID TISSUE

    • MORPH:
    • --DIFFUSE (not follicular) EFFACEMENT BY MONOTONOUS INFILTRATE OF SMALL LYMPHs
    • --SMALL LYMPHs NOT CLEAVED
    • --PSEUDO GROWTH CENTERS

    • FEATURES OF LOW GRADE LYMPHOMA
    • --LOW MIT
    • --ABSENT NECROSIS

    • GENETICS:
    • DEL OF 11q, 13q14.3, AND 17p
    • --TRISOMY 12q
    • --NO CD10

    • PROG:
    • --LOW GRADE INDOLENT IN MOST
    • --DEL 11q AND 17p WORST
    • --5-10% RICHTERs TRANS TO LARGE CELL LYMPHOMA
    • --15-30% PROLYMPHOCYTIC TRANS IN BLD
  115. BURKITT LYMPHOMA
    30% OF KID NHL

    SUBSET IN HIV Pts

    MOST TUMORS MANIFEST IN EXTRANODAL SITES

    • AFRICAN (ENDEMIC)
    • --TROPICAL EQUATORIAL & NEW GUINEA
    • --MEAN AGE 7 yrs
    • --MAXILLA, MANDIBLE, OVARIES, KIDNEYS

    • NON-AFRICAN (SPORADIC)
    • --NON ENDEMIC AREAS
    • --11 yrs
    • --ILEOCECUM, PERITONEUM

    • MORPH
    • --LOSS OF nL ARCH
    • --DIFFUSE LYMPHOID INFILT
    • --MEDIUM SIZE CELLS
    • --ROUND NUC (small noncleaved cells)
    • --MOD BLUE CYTOPLASM
    • --TINGIBLE BODY MACROs AND HIGH MIT ACT, STARRY SKY APPEARENCE

    • IMMUNOPHENOTYPE
    • --B CELL CD 19, 20
    • --MONOCLONAL (kappa or lambda)
    • --CD 10
    • --BCL-2 NEG (follicular is +)

    • PATHOGENESIS
    • -- C-MYC (8q24) TRANS
    • --t(8;14), (8;22), or (8;2)
    • --EBV RELATED (95% in african, 15% in non-african)

    • CLINICAL
    • --AGGRESSIVE HIGH GRADE TUMOR
    • --CURABLE
  116. MALT LYMPHOMA
    • MARGINAL ZONE LYMPHOMA
    • --HETEROGENEOUS
    • --SPLEEN, LYMPH, EXTRALYMPH

    • ASSOC w CHRONIC INFLAM DISORDERS RELATED TO INFECTION OR AUTOIMMUNE
    • --HELICOBACTER PYLORI
    • --SJOGREN, HASHIMOTO THYROIDITIS

    • CLINICAL PRESENTATION EXTRANODAL
    • --STOMACH MOST COMMON
    • --SALIVARY, LUNG, ORBIT, SKIN

    • MORPHOLOGY
    • --INFILTRATE OF CENTROCYTE-LIKE CELLS, MONOCYTOID B-CELLS, PLASMA CELLS (MIXED)
    • --REACTIVE BENIGN GERMINAL CENTERS
    • --LYMPHOEPITHELIAL LESIONS (infiltrate destroy epi of crypts, ducts, and glands)

    • IMMUNOPHENOTYPE
    • --B CELL CD 19, 20
    • --MONOCLONAL SIg
    • --NEG CD 5 & 10

    • GRADE
    • --LOW
    • --LOCALIZED, STAGE I or II

    • ETIOLOGY
    • --GASTRIC MALToma
    • --ASSOC w HELICOBACTER INF

    • CLINICAL COURSE
    • --INDOLENT
    • --POSS CURE WITH ANTIBIOTICS
    • --IF GENETIC ABN (ex t(11;18)), NO LONGER CURE w ANTIBIOTICS
  117. MYCOSIS FUNGOIDES
    MATURE T-CELL LYMPHOMA ARISING IN SKIN

    • CLINICAL FEATURES
    • --MOST COMMIN CUTANEOUS T-CELL LYMPHOMA
    • --INDOLENT WAXING AND WANING, 8-9 yrs
    • --PROGRESSIVE SKIN LESIONS FROM RASH TO PLAQUES, TO TUMOR
    • --SEZARY SYND: PERIPH BLD INVOLVEMENT

    • MORPH
    • --LYMPHOID INFILTRATE
    • --SMALL & LARGE CELLS w CEREBRIFORM NUC IN EPI
    • --PAUTRIER'S MICRO ABSCESSED

    • IMMUNOPHENOTYPE
    • --MATURE HELPER T-CELLS
    • --CD 4, 3

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