Umbilical cord blood transplantation induces a durable remission in hepatosplenic gamma‐delta T cell lymphoma with associated hemophagocytic lymphohistiocytosis

Abstract

Thirty patients (88%) died, with a median OS of 4.5 months (95%CI 2.6-8.6) and a 3-year OS of 18% (95%CI 7-32). Cause of death was disease relapse/progression in 23 cases (77%), while the remaining 7 (23%) died of nonrelapse mortality (NRM). Twenty-nine patients were evaluable for response after SCT, as five died of early NRM. Nineteen patients (66%) achieved CR: 13/19 (68%) relapsed after a median of 3.4 months (range 1.7-18.2) and survived for a further 1.4 months (range 0.4-9); among the six patients in sustained CR, four are alive and two died of NRM after 79 and 55 months. Ten patients (34%) had resistant leukemia after SCT and died in a median of 2.5 months (range, 1.4-6.6).Considering pretransplant characteristics, OS was not predicted by gender, karyotype, age at SCT, time to SCT, number of chemotherapy courses, donor type, stem cell source, or conditioning regimen. There was a significant association between OS and WBC count at SCT (<10,000/ml: 20 months [95%CI 4.6-NA] vs. 10,000/ml: 3.6 months [95%CI 1.9-4.5], P < 0.0001), platelet (PLT) count at SCT (>30,000/ml: 18.8 months [95%CI 3.6-NA] vs. 30,000/ml: 3.7 months [95%C 1.9-10], P 5 0.05) and PB blasts at SCT (absent: 79 months [95%CI 2.6-NA] vs. present: 4.5 months [95%CI 3.4-8.6], P 5 0.03). Stratifying patients according to WBC, PLT, and circulating blasts, we identified four groups with different survival: median OS for patients with 0, 1, 2, or 3 adverse prognostic factors was 79, 10.1, 4.1, and 3.5 months, respectively (P 5 0.004) (Fig. 1).Our data confirm that allogeneic SCT can cure a minority of patients with primary refractory AML, and that relapse is the major cause of treatment failure after SCT. In our experience, 3-year OS was lower than that of the MDACC group (18% compared to 39%) but comparable to that reported by the GITMO (10%) [2] and significantly superior to survival after salvage chemotherapy (2-5%) [1,3]. Jabbour et al. found higher WBC, lower PLT, and higher BM blasts percentage at SCT to be associated with superior OS, but did not define any cutoff value [1]. In the setting of unrelated donor transplant for AML primary refractory to various induction strategies (conventional 3 1 7 or HDACbased regimens), Craddock et al. identified number of chemotherapy courses before SCT (>2) and BM blasts percentage (above a median of 38.5%), along with patient CMV serostatus, as predictive factors for OS [4]. We found that WBC count <10,000/ml, PLT count >30,000/ml and absence of circulating blasts predict superior long-term outcome after SCT. If confirmed in larger studies, this finding may help in designing a simple pre-SCT score to identify AML patients primary refractory to intensive induction chemotherapy that could maximally benefit from the transplant procedure.

Transplantation in Rare Lymphoproliferative and Histiocytic Disorders

“…50,51 At the Moffitt Cancer Center, a 22-year-old woman presented with hepatosplenomegaly, fever, and severe hypotension. 52 Bone marrow biopsy and pathology from splenectomy showed the presence of γ/Δ hepatosplenic T-cell lymphoma and HLH. Upon presentation, her ferritin level was 20,700.…”