73 yrs old male underwent threpine biopsy due to suspition of LPL (WM).
CLINICS: Consultation case due to anemia. Proteinemia 120 g/l, ~ 50% M gradient.
HISTO: Cellularity 90%, up to 60% difusse lymphoid infiltration with Ig inclussions. additionally- coarse plasma cell clusters up to 20%. No fibrosis.
IH: CD21/CD23(-)(FDC absent, single lymphos CD23+); CD20(+/++)(heterogenous) 80%; cyclinD1(++)(brand. r-ja)(up tp 20- 25% in probably plasmoid cells); CD79a(+/+++) 100% (plasma cells more prominent); IgM(+/+++) 100% (both populations); IgD(-)(pavieniai limfocitai silpnai +); CD43(-)(granuliopoezė +);
CD3/CD5+ small medium interstitial lymphos;
CD138/CD38+ plasmoid infiltrates up to 20%: CD56(-); IgG+ singles only; Ig kappa(+/+++) >> Ig lambda; CD117 (-)(mastocytes +).
MOLECS and FLOW (BM): pending;
TREATMENT: a) plasmapharesis; b) hemotransfussion; c) CycloPhos+ steroids; d) throbo-prophylaxis, ABs, hyperuricaemia correction. IgM going down, slight lymphocytosis in blood transforming into lymphopenia;
PROPOSAL: DIFFICULT TO CLASSIFY:
a) B NHL with plasmoid diff., 60% BM. IH: CD20/CD79a/Pax5+; LCA+; CD138/CD38/CD23/CyclinD1(-); IgM+; IgG/IgD(-); Ig kappa/lambda(-)(?);
b) Plasmoid hyperplasia, 20% BM with IH: CD20/CD79a+; CD138/CD38+; IgM+; LCA+; Pax5(-)(?); Ig kappa+; CyclinD1+; IgD/IgG(-).
FLOW and molecular evaluation recommended(MYD88? subpopulation clonal analysis?).
Comments:
1. CyclinD1+ not compatible with WM, but general IH fits.
2. MM lymphoplasmacytic variant: not compatible due to focal CD138+; IgM+; IgG(-);
3. 2 diseases possible?: B LPL (WM) ir MGUS/MIELOMA?