Non-neoplastic hematopathology_hemepath boards

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Non-neoplastic hematopathology_hemepath boards
2014-09-21 01:13:12
Non neoplastic hematopathology fellowship hemepath

Non-neoplastic hematopathology hemepath boards
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  1. What benign condition can show aberrant expression of CD34 in megas?
    Megaloblastic anemia
  2. Characteristic PB, BM and clinical findings in megaloblastic anemia?
    Sieve-like nucleus, pancytopenia, marked anisopoikilocytosis (from spleen chomping up RBCs)
  3. Characteristic findings in spleen with ITP?
    • 1. Secondary follicles with well developed GCs in white pulp (follicles diminished w/steroid tx)
    • 2. Dilated sinuses
    • 3. GC contain CD41 and show macrophage phagocytosis of nuclear debris
    • 4. Mild myeloid metaplasia or extramedullary hematopoiesis (megas mainly)
    • 5. Histiocytes and neutrophils in red pulp
  4. Name causes of extramedullary hematopoiesis.
    • 1) MPD: Idiopathic Myelofibrosis, polycythemia vera, CML
    • 2) Carcinoma with mets to bone
    • 3) MDS
    • 4) Hepatitis
    • 5) Hemolytic anemia
  5. CD117
    myeloblasts and will also highlight proerythroblasts, mast cells, and occasionally promyelocytes.
  6. IgG4 disease findings
    • 1) LPC infiltrate with lots T cells
    • 2) fibrosis
    • 3) phlebitis
    • 4) elevated serum IgG4
    • 5) esp middle aged+ and males
    • 6) can present as masses or nodules in lung
  7. In HIV, what bone marrow features are prominent?
    • 1) Chronic thrombocytopenia (impaired production and immune-mediated peripheral destruction)
    • 2) small hypolobated or denuded megakaryocytes with pyknotic/smudgy nuclei
    • 3) megakaryocytic hyperplasia and dysplasia
  8. What is the theorized selective advantage in PNH?
    Clonal abnormalities of GPI-linked surface proteins confer increased resistance to NK cell mediated apoptosis, increasing survival in an adverse enviroment of an aplastic BM

  9. Identify the image.  What conditions cause this?
    Cabot ring - microtubules, remnants from mitotic spindle; pernicious anemia, erythroid production disorder, Pb poisoning
  10. All of the following are major extrinsic causes of neutropenia except:
    A) Drugs
    B) CAD
    C) Infections
    D) Autoimmune disorders
    E) T-LGL leukemia
  11. What is the differential diagnosis for a non-neoplastic sinus pattern in a LN?
    Rosai Dorfman vs. lymphangiogram effect vs. nonspecific/reactive vs. Whipple disease hemophagocytic syndrome vs. dermatopathic lymphadenitis
  12. What is the differential diagnosis for a non-neoplastic follicular pattern in a LN?
    Viral (HIV) vs. Toxoplasmosis vs. syphilis vs. Castleman disease vs. rheumatoid arthritis or Sjogren syndrome vs. nonspecific
  13. What is the differential diagnosis for a non-neoplastic inrterfollicular/paracortical pattern in a LN?
    Viral (mono, CMV, postvaccine) vs. hypersensitivity (dilantin) vs. Kimura disease (eosinophilia with florid follicular hyperplasia and immunoblasts)
  14. What is the differential diagnosis for a non-neoplastic granulomatous pattern in a LN?
    • -Suppurative granulomata (cat-scratch, lymphogranulomatous venereum, tularemia)
    • *LGV looks like cat-scratch in an inguinal node
    • -Necrotizing (TB, Histoplasmosis, C. immitus, M. scrofulaceum)
    • -Kikuchi (karyorrhexis with histiocytes and absent neutrophils)
  15. What is the differential diagnosis for a non-neoplastic diffuse pattern in a LN?
    Mainly viral (EBV, CMV, measles, post vaccine)
  16. If a person presents with thrombocytopenia, left-shifted neutrophils without toxic granulation, hemoconcentration, and >10% immunoblast, what virus should you be worried about?
  17. What does the presence of osteoblasts/osteoclasts in an adult BM indicate?
    Bone destruction/repair (usually pathologic)
  18. What are the clinical findings in hemophagocytic syndrome?
    • 1) Hepatosplenomegaly
    • 2) Markedly increased ferritin (>500 microg/L), CRP, ESR
    • 3) Pancytopenia
    • 4) Hemophagocytosis
    • 5) Low fibrinogen
    • 6) Elevated LFTs
    • 7) Fever
  19. Typical causes of hemophagocytic syndrome?
    EBV, T-cell lymphoma, autoimmune

  20. BM biopsy. What is the likely infection here?
    Q fever (ring granuloma)

  21. What's this?
    Pappenheimer bodies - blue/purple in color, in periphery, and not perfectly round; smaller than Howell-Jolly bodies; expelled iron-containing mitochondria
  22. All of the following can show extensive replacement of BM by fibrous/spindled processes except:
    A) Hodgkin lymphoma
    B) AITL
    C) Hairy cell leukemia
    D) AML M4
    E) Mastocytosis
    D - only AML M7 (megakaryoblastic)
  23. What are potential causes of serous fat atrophy (gelatinous transformation)?
    • Mnemonic: MaDAM anorexic
    • Malnutrition - anorexia
    • Debilitation
    • Advanced malignancy
    • Malabsorption
  24. What is the average MPV for a platelet?  What is the relationship between platelet volume (MPV) and platelet count?  When does MPV go down? Up?
    Between 2fL and 20fL; inverse relationship; down with thrombocytopenia or impaired production (AA); up with destruction, ITP, MPN, Bernard Soulier, etc. (basically getting bigger immature plts pushed out)

  25. a 36 year old female presented with fever and flank pain. She was found to be pancytopenic with a WBC of 1.7, K/mm3. Absolute neutrophil count of 1.1, H/H 11.6/33.3, and plts 124 K/mm3.  Diagnosis?
    Parvovirus (picture shows giant proerythroblasts with nuclear inclusions)
  26. A two-month old girl presented with cough and SOB. CBC showed leukocytosis with marked lymphocytosis.  PB smear below. Dx?
    Pertussis -Numerous mature lymphs with clefted nuclei

  27. Likely PB smear findings?
    (Megaloblastic anemia with sieve-like chromatin) - macrocytic anemia with hypersegmented neutrophils

  28. Metabolic disorder?
    Nieman Pick - vacuolated cells

  29. Metabolic disorder?
    Gaucher's disease -  tissue paper looking
  30. What is the defect in Autoimmune lymphoproliferative syndrome (ALPS)?  Flow cytometric findings? Clinical presentation?
    • Defect in Fas/CD95/Apo-1 mediated apoptosis; CD4-/CD8-, CD25-, CD45RO-, CD3+, CD57+
    • LAD and splenomegaly in healthy child progresses to cytopenias
  31. Sequence of abnormal lab values in iron deficiency anemia with respect to ferritin, transferrin, serum Fe, ZPP, and MCV?
    ↓ferritin → ↓serum Fe →  ↑ZPP→ ↓Hb → ↓MCV