Biphenotypic extramedullary blast crisis as a presenting manifestation of Philadelphia chromosome-positive CML in a child.

A 14-year-old child presented with generalized lymphadenopathy and massive hepatosplenomegaly. Peripheral smear and bone marrow examination were suggestive of Ph' positive chronic myeloid leukemia (CML) in chronic phase. However, lymph node biopsy showed extramedullary blast crisis with evidence of myeloid and T cell markers in blasts. Reverse transcriptase-polymerase chain reaction from lymph node aspirate revealed transcript for bcr-abl p210. Thus, we present here a unique case of childhood CML with extramedullary biphenotypic blast crisis (myeloid/T cell type) at initial presentation with bone marrow remaining in chronic phase. This case provides further evidence to the highly heterogeneous presentation of CML.

Acute basophilic leukemia with t(8;21).

Acute basophilic leukemia (ABL) is a rare form of leukemia. The diagnostic criteria have recently been described. Morphological evidence for basophilic lineage is required for its classification. However the criteria for remission status and standard therapy is not established. Here we have described an atypical case of ABL and reviewed the literature to high light issues regarding diagnosis and management, which need further discussion.

High dose chemotherapy followed by autologous haemopoietic stem cell transplant in multiple myeloma.

BACKGROUND: High dose chemotherapy followed by autologous stem cell transplant is currently used for the treatment of patients with advanced multiple myeloma. However, there are no reports of the results of this treatment modality in Indian patients. METHODS: Fifty patients with advanced multiple myeloma underwent treatment with high dose melphalan followed by autologous stem cell transplant (bone marrow: 7; peripheral blood stem cells: 43). The patients' ages ranged from 26 to 65 years (median: 52 years) and 35 were men. All patients had received chemotherapy initially with a mean of 9.4 cycles (range: 1-36). Thirty patients had evidence of chemosensitive disease at the time of transplant. The mean interval from diagnosis to transplant was 17.5 months (range: 3-129 months) and the median number of mononuclear cells infused was 4.86 x 10(8) per kg (range: 2-10.48). RESULTS: Post-transplant, 43 of 50 patients engrafted. The median number of days to engraftment (absolute neutrophil count > 500/cmm) was 12 (range: 9-24) and to achieve platelet transfusion independence (> 20,000/cmm) was 13 (range: 8-36). Seven patients died prior to engraftment. Grade III-IV oral mucositis was the major non-haematological toxicity. Excluding the 4 patients who had complete response prior to the transplant and continued in the same status post-transplant, 31/46 patients (67%) responded; complete response was achieved in 25 (54%) and partial response in 6 (13%). Patients with chemosensitive disease had higher rates of complete response; 20 of 26 patients with partial response at transplant achieved complete response compared to 5 of 20 patients with persistent/refractory disease (p < 0.01). Currently, 34 of 50 (68%) patients are alive, 17 (34%) disease-free, 6 with disease are on salvage therapy, 11 (22%) with positive monoclonal protein but asymptomatic are under observation. Nine (18%) patients have died; 8 due to progressive disease and 1 of an unrelated cause. The median follow up for the entire group is 26 months (range: 1-144 months). The Kaplan-Meier probability of overall and progression-free survival for the whole group at 30 months is 62% +/- 8.11% (SE) and 42% +/- 9.54% (SE), respectively. A haemoglobin level < or = 10 g/dl (p < 0.003) affected the survival adversely. Chemosensitive disease (p < 0.008) at transplant and complete response post-transplant (p < 0.0001) were associated with significantly longer survival. CONCLUSION: High dose melphalan followed by autologous stem cell transplantation is an effective treatment for patients with advanced multiple myeloma and achievement of complete response is associated with improved survival.