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Plasma cell leukemia- case presentation. 5/2008. 45 year old woman 11/07 IGA Kappa MM – TP120, ESR 120, Ca 2.9 mmol/L, Creatinin 97 micromol/L, splenomegaly(17cm), multiple lytic lesions. IGA 3680 mg/dl, Alb 37g/L, β 2 microgl. 5800 mg/ml Elevated LDH 765 U/L
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45 year old woman • 11/07 IGA Kappa MM – TP120, ESR 120, Ca 2.9 mmol/L, Creatinin 97 micromol/L, splenomegaly(17cm), multiple lytic lesions. • IGA 3680 mg/dl, Alb 37g/L, β2 microgl. 5800 mg/ml • Elevated LDH 765 U/L • Blood count – HgB 6.5 Plt 45,000 • BM- consistent with plasma cell myeloma (aspiration- dry tap) • Cytoginetics- hyperploidity, t(14;20) • Fat pad and BM negative for Amyloid deposits • HD Dexamethasone and Pamidronate – TP dropped to 104 and calcium to normal levels
Thalidomide was started and the patient was hospitalized due to sever abdominal pain • 1/08 splenomegaly(22cm) with normal flows, pancytopenia, TP 107 • Peripheral blood – plasma cell leukemia(>20% plasma cells) • BM-replaced with atypical plasma cells
27/1/08 Cyclo 300mg/m2 (d 1,8) Dexa 40mg(1,2,4,5,8,9,11,12) Velcade 1.3mg/m2 (D1,4,8,11) • A week after starting chemotherapy- hypercalcemia, TP120 • Melphalan 35 mg on D1 and D7(11-18/2/08), Thalidomide, Velcade and Dexamethasone were continued. • Spleen continued to enlarge, hypercalcemia, TP, hyperbilirubinemia
16/3/08 – DT PACE reduced dose • Improvement in spleen and liver size, bilirubin, TP, calcium • LLL pneumonia • She was found to have a full matched sibling donor • 10/4/08 DT PACE full dosage • On termination of chemotherapy –sepsis • Liver and spleen enlargement, sever hyperbilirubinemia, ascites, bleeding tendency, liver encephalopathy leading to her death on the 29/4/08
Dilemmas • Primary vs. Secondary Plasma Cell leukemia • The best treatment options in Plasma Cell leukemia – Thalidomide, Velcade, Revlimid, upfront auto transplantation or allogeneic transplantation
Dilemmas Treatment options • Thalidomide • Velcade • Revlimid • ASCT / Allo-SCT