Pathology Associates Of Lexington, P.A.
Pathology Associates Of Lexington, P.A.
Pathology Associates Of Lexington, P.A.
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Hematopathology Topics
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  • CBC (complete blood count test) topics.
  • Thymic/thoracic
  • Bone marrow findings
  • Coagulation
  • spleen findings:
    1. siderosis: implies hemolytic anemia or hemochromatosis [L07-6908].
    2. increased foamy macropahges: implies platelett consumption by spleen as in ITP.
    3. increased red pulp polys: implies sepsis..."septic splenitis" (or prolonged operative manipulation).
    4. enlarged & inflammed spleen (acute, subacute, or chronic splenitis [likely related to some systemic infection...FA08-32]; acute or chronic splenic tumor or pseudotumor; septic spleen): is enlargement primarily congestion?...if so, would not use the tumor or pseudotumor term.
    5. increased red pulp fibrosis: implies increased portal forward or back pressure.
  • benign pathological lymph nodes:
    1. infectious:
      • suppurating stellate granulomatous adenitis:
        1. cat scratch (Bartonella): whole node often destroyed [L07-2437].
        2. LGV (Chlamydia).
        3. tularemia: likely quite ill.
        4. always check for mycobacterial.
      • casseating granulomatous lymphadenitis: AFB or fungal infections.
      • hyperplasia with prominent & serpigenous germinal centers: think viral.
    2. noninfectious (at least no organism demonstrated):
      • stellate granulomatous, nonsuppurative: think of rheumatoid problems; when optimally processed and no serological, culture, or PCR evidence of causative organism, default DX is "idiopathic necrotizing granulomatous lymphadenitis" [L08-4147].
      • mesenteric: at autopsy, one fairly frequently notes some small bowel mesentery nodularity...especially in thick, fatty mesenteries...which has a tan-yellowish cut surface involving fatty nodes with some orange perinodal fatty discoloration..."nodular mesenteric adenopathy".
      • foreign material:
        1. silicone adenopathy [L07-8846].
        2. joint prosthetic "wear & tear debris" detritic lymphadenopathy8 which tends to enlarge by sinus histiocytosis, silicone & polyethylene debris refractile on polarized light exam & metallic debris causing black nodes.
        3. oleogranulomatous lymphadenopathy: in abdominal nodes & due to oral ingestion of waxy lipids as part of the cooking process [L08-12403 cystic duct node].
      • suppurating stellate granulomatous splenitis:
        1. infectious, as above, but incompletely worked up
        2. idiopathic necrotizing granulomatous lymphadenitis (intense study is negative for organisms known to cause this reaction)[L08-4147].
    3. other:
      • primarily lymphoid enlargement:
      • primarily nonlymphoid enlargement:
        1. medullary stromal elements increased:
          1. fibrovascular, cause unknown [L09-5338].
          2. detritic sinus/medullary histiocytosis related to orthopedic prostheses.
  • Pseudolymphoma (vs. not-as-yet-diagnosable lymphoma):
    1. skin: [MMM, S-05-6780; HAB, S-06-16018...use of CD3, CD20, & CD23 (also maybe CD5, CD4 & CD8) to show a benign, non-monotypic immunoarchitectural pattern]. [S09-7686 recurring skin of head & neck lesions of pseudolymphomatous perifolliculitis] Lymphoma would have a more monotypic study by IHC markers.
    2. nodes:
      • in a case of "common variable immunodeficiency (compensatory increase in T-cell component) [LMC-05-6145].
    3. lung:
      • in a case of "common variable immunodeficiency (compensatory increase in T-cell component) [LMC-05-6145].
    4. other: Castleman's disease [L09-5594] B-cell tumor.
  • Malignant lymphoma:
    1. Hodgkin's disease:
      • lymphocyte predominant can be very subtle, even failing to show atypical histiocytes or R-S cells [___].
    2. non-Hodgkin's lymphoma
  • Leukemia:
    1. acute: hypercellular marrow & peripheral blasts may be absent (aleukemic), or at low levels (subleukemic [L09-3953])
      • lymphoid
      • monocytoid: spectrum of with or without "differentiation" [S-04-5214].
      • granulocytic (AML...blast count >30%)
      • mixed: AMML
      • other (mast cells, plasma cells, megakaryocytes, lymphoma cells)
    2. hypoplastic acute: hypocellular marrow with increased blasts; peripheral blood may or may not be blastic. If thought benign & given GCSF and Procrit, will dump increased blasts into PB because these agents cannot make leukemic blasts mature5.
    3. smoldering: hypercellular marrow
      • lymphoid
      • monocytoid
      • granulocytic [S-03-4109 ].
      • other (mast cells, plasma cells, megakaryocytes, lymphoma cells).
    4. subacute: hypercellular marrow
      • lymphoid
      • monocytoid
      • granulocytic
      • other (mast cells, plasma cells, megakaryocytes, lymphoma cells)
    5. chronic: hypercellular marrow
      • lymphoid: adult T-cell lymphoma/leukemia [S07-2516]
      • monocytoid
      • granulocytic (CGL, CML): 95% have the Philadelphia chromosome gene rearrangement by reciprocal translocation between 9 & 22 and located on 22. Surveilence during therapy or post transplant is by FISH probes to BCR/abl (sensitive to about 1 in a 100 cells) or by RNA PCR amplification for the BCR/abl genetic abnormality (sensitive at between 1 in 1000 to 1 in 1,000,000 cells)...in early 2009, "FISH negative" indicates "remission" (or continued remission [L07-5759]) despite any positivity of PCR (tho such positivity stands as a clue to increased risk that remission may come to an end).
      • other (mast cells, plasma cells, megakaryocytes, lymph oma cells)
  • Myeloproliferative syndromes (MPS or CMPD): leukocytosis work up; MPS/CMPD is a hypercellular marrow with some effectiveness in raising counts of one or more peripheral blood elements; the JAK2 gene related to tyrosine kinase is altered as a point mutation into an oncogene & is strongly specific for MPS [S08-3992] (JAK2V617F mutations are present in almost all patients with polycythemia vera, and in approximately half of those with essential thrombocytosis and myelofibrosis). The old LAP score is an obsolete test, though still useful as an on-site proxy test for the Ph1 chromosome or the BCR/ABL gene mutation (on chromosome 9, the "abl" gene can translocate onto chromosome #22 at the "BCR" gene & make a "BCR/abl gene" mutation which then causes the cells to release tyrosine kinase which drives the stem cells to produce too many WBCs [95% of CMLs have this 9/22 translocation]).
    1. polycythemia vera (Osler–Vaquez disease).
    2. thrombocytosis: reactive vs. essential thrombocythemia (ET) vs. pre-polycythemic phase of PV...the role of JAK2 & marrow morphology in the DDX
    3. chronic idiopathic myelofibrosis/myelosclerosis (agnogenic myeloid metaplasia)
    4. chronic myelogenous (myeloid) leukemia
    5. atypical chronic myelogenous (myeloid) leukemia
    6. chronic neutrophilic leukemia
    7. chronic myelomonocytic leukemia, myeloproliferative variant
    8. juvenile chronic myeloid leukemia
    9. chronic eosinophilic leukemia (and the hypereosinophilic syndrome)
    10. MPS/CMPD, unclassifiable
  • Myelodysplastic syndromes (MDS): hypercellular marrow with some ineffectiveness in one or more peripheral blood elements so that there are usually -cytopenias at presentation
    1. primary (the blast % is based on morphology because N/C asynchrony shows elevated blast markers sooner than blastic nuclear change2 [S-03-12958]):
      • refractory anemia (RA) [S-04-3702].
      • refractory anemia with ringed sideroblasts (RARS)
      • refractory anemia with excess of blasts (RAEB)...blasts <20%
      • refractory anemia with excess of blasts in transformation (RAEB-T)...blasts 20-30%; new WHO advocates dropping this acronym and calling this "AML >20%".
      • chronic myelomonocytic leukemia (CMML)
      • chronic myelomonocytic leukemia in transformation (CMML-T)
      • myelodysplastic syndrome, unclassified (MDS-U)
        1. megakaryocytic type [S-03-15566] (meg-only dysplasia)
      • "5Q- syndrome": predominantly in elderly women and consists of macrocytic anemia, thrombocytosis (50% of patients), erythroblastopenia and megakaryocyte dyspoietic hyperplasia with nuclear hypolobation & chromosome 5q deletion.
    2. secondary (therapy/toxic related)
  • MPS/MDS overlap cases: [S-03-12900]
  • Other:
    1. cytokine effect in bone marrow [LMC-03-7297].
    2. interleukin-6 syndrome [S-03-16214].
    3. myeloid hyperplasia of uncertain significance (especially appropriate when hyperplasia is "central medullary" 7 spares paratrabecular areas [S09-4139]).
  • Monoclonal cell populations & marrow may be histologically normal: molecular monoclonality before morphologic or clinical criteria are met for a malignant process (we'd categorize sort of like we do small monoclonal proteins as MGUS). In marrows for MGUS workups, we are trying to (1) see if we can demonstrate morphological evidence of increased plasma cells; and, (2) do we see any concerning cytological features ?; or (3) are there frankly malignant features? It often takes the IHC marker for CD138 to help one discern a too-great concentration of plasma cells (but CD138 makes the percentage some higher than truly is). Failure to discern an abnormal morphological population in these tiny samples DOES NOT rule out plasma cell dyscrasia.
References:
  1. Brunning RD & McKenna RW, Atlas of Tumor Pathology: Tumors of the Bone Marrow, AFIP 3rd series fascicle #9, 1994.
  2. Am. J. Heme, Sept. 2003.
  3. Tumors of Haematopoietic and Lymphoid Tissue, WHO, 2001.
  4. Dr. Armstrong's Jan. 2005 memo (ref. AJCP [Dr. R. W. McKenna] Oct. 2004, p. 588).
  5. Our hemepath's or consultant's tips & comments.
  6. Goldman, John M., "A Unifying Mutation in Chronic Myeloproliferative Disorders", NEJM 352(17):1744-1746 April 28, 2005.
  7. Gianelli U., et. al., "The Significance of Bone Marrow Biopsy and JAK2V617F Mutation in the Differential Diagnosis Between the 'Early' Prepolycythemic Phase of Polycythemia Vera and Essential Thrombocythemia", AJCP 130(3): 336-342 September 2008.
  8. Benz EB, et. al., "Lymphadenopathy Associated with Total Joint Prostheses. A Report of Two Cases and a Review of the Literature", The Journal of Bone and Joint Surgery 78:588-93 (1996).
(posted 2002; additions 19 April 2003; latest addn 5 July 2009)
 
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