- maturation arrest
- long term tx - transplant, allogenic bone marrow graft
- Shh config with genetics as final dx?
- 50% hypercellular, 25% normacellular, 25% hypocellular
- increased reticulin
- primary - 70 y/s old elderly, typically present with fatigue
- secondary - chemo/radiation,
- can progress to AML (1/3 eventually -> AML)
- cytogenetics
- chromosome deletion
- 5q, 7q, or 20q, 12p
- types
- refractory anemia
- refractory anemia with ringed sideroblasts
- refractory anemia with excess blasts (RAEB)
- chronic myelomonocytic leukemia
myeloid maturation, R shifted, e.g. - infection
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myelofibrosis - a) myeloid dysplasia or b) acute myelofibrosis
apperance of extramedullary hematopoiesis or malignant myeloid cells in another organ, usually spleen, with fibrosis in the marrow
increased reticulin is present around clusters of megakaryocytes; megakaryocytes have aberrant N/C ratios and hyperchromatic, bulbous or irregularly folded nuclei
primarily adults - (mean age 60), younger when follows polycythemia vera or CML
marrow - three stages - hypercellular
- patchy fibrosis
- obliterative myelosclerosis
indolent, prolonged course, median survival 10 yrs
myelofibrosis staging by reticulin
0-1 normal
normal bone marrow should have CD3 > CD20
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MALT (extranodal marginal zone lymphoma)
- common cause of MALT in stomach, H-pylori
- although classified as a cancer(and clonal), when the insult is removed, early MALT disappears (even when there's bone marrow involvement)
- however, late MALT will stay
- reactive follicles with germinal centers and plasma cells
- t(11;18)(q21;q21), predictive of poor response to eradication therapy.
AML
- gelatinous / myxoid
diffuse large b-cell lymphoma (DLBL) - CD3 <>
follicular lymphoma -> progress to DLBL
can present synchronous
progression of FL to DLBL may involve p53 mutation
if BM involved by FL, better prognosis at stage 4
if BM involved by DLBL, worse prognosis at stage 4
p53
anti-oncogene
a negative regulator of cell cycle
activates p13, which inactivates cyclins
normally present in low level with short half life loss of function mutants have an increased half-life; levels accumulate to 5-100 x normal
also seen in, colon, breast, lung, leukemia, sarcomas
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question: why stain for CD20 and Pax-5 when both stain for B cells?
treatment for follicular lymphoma is anti-CD20 antibodies (rituximab)
if pt treated it and there's still residual disease, then they will be CD20(-), so use Pax-5 to stain
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immunostains
CDX2
- intestine
- nuclear stain
CD34
- blast, progenitors, or block in maturation, vascular endothelium
CD117 - stains for mast cell or stem cell, stem cell has higher N/C ratio, less intense staining than mast
CD61 - megakaryocytes, platelets
CD30 - activated T, B, Reed-Sternberg cells
reticulin - around blood vessel don't count, also abundant in lymphoid aggregate
glycoporin - erythrocyte stain
PAX-5 - pan B-cell marker
