| Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access |
| Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc |
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Case Report
Volume 11, Number 8, August 2020, pages 256-261
Adult Lymphoma-Associated Hemophagocytic Lymphohistiocytosis: A Clinical Case Series in a Predominantly Hispanic Cohort
Tables
| Case | Age/sex | HLH criteria | Associated malignancy | Stage | Interval time between NHL and HLH | NHL therapy | NHL response | HLH treatment | Outcome/status | Survival from HLH diagnosis |
|---|---|---|---|---|---|---|---|---|---|---|
| CR: complete remission; DLBCL: diffuse large B-cell lymphoma; HD-MTX: high-dose methotrexate; HLH: hemophagocytic lymphohistiocytosis; RD: refractory disease; ND: not determined; PR: partial response; NHL: non-Hodgkin lymphoma; PCNSL: primary CNS lymphoma; PTLD-ALCL: post-transplant lymphoproliferative disorder-anaplastic large cell lymphoma; ALK: anaplastic lymphoma kinase; SCT: stem cell transplantation; EBV: Epstein-Barr virus. | ||||||||||
| 1 | 53/F | Fever, splenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, elevated sCD25 | Peripheral T-cell lymphoma, NOS | II | HLH diagnosed at second lymphoma relapse, 36 months after initial lymphoma diagnosis | CHOP, salvage ICE followed by autologous SCT, GEM/OX on 2° relapse | CR to all three lines of therapy | HLH-2004 regimen then allogeneic sibling SCT | Died 13 days after allo-SCT due to massive cerebellar infarct | 298 days |
| 2 | 51/F | Fever, splenomegaly, cytopenias, hemophagocytosis, hypertriglyceridemia, hyperferritinemia | Peripheral T-cell lymphoma, NOS | PCNSL | Concurrent | CHOP-based regimen plus HD-MTX | ND | Treatment of underlying malignancy | Died after cycle 1 of CHOP due to septic shock | 56 days |
| 3 | 54/M | Fever, splenomegaly, cytopenias, hemophagocytosis , hypofibrinogenemia, hyperferritinemia | EBV+, extranodal NK-/T-cell lymphoma of the intestine (right colon, ileum) | IE | HLH diagnosed 11.9 weeks prior to NHL | NA | RD | HLH-1994 regimen | Died in 4 months with refractory disease | 120 days |
| 4 | 55/F | Fever, splenomegaly, cytopenias, hemophagocytosis , hypertriglyceridemia, hyperferritinemia | EBV+, PTLD-ALCL, ALK- | IE | HLH diagnosed 12.6 weeks after NHL | Topical nitrogen mustard | CR | HLH-2004 regimen | Alive in remission | 1,572 days as of last follow-up |
| 5 | 70/F | Fever, splenomegaly, cytopenias, hemophagocytosis, hypertriglyceridemia, hyperferritinemia | DLBCL, NOS | IV | Concurrent | Vincristine/cyclophosphamide/methylprednisolone followed by R-CEOP | RD | Treatment of underlying malignancy | Died under hospice care due to refractory disease | 50 days |
| 6 | 52/M | Fever, splenomegaly, cytopenias, hyperferritinemia, hemophagocytosis, hypofibrinogemia, elevated IL-2R | EBV+, DLBCL | IVBE | Concurrent | Reduced dose cytoxan (five doses) + rituxan (two doses) followed by R-CHOEP + IT MTX for two cycles, then DA-REPOCH for four cycles | CR | Treatment of underlying malignancy | Alive in remission as of last encounter | 296 days as of last follow-up |
| 7 | 73/F | Fever, splenomegaly, cytopenias, hemophagocytosis, hypertriglyceridemia, hyperferritinemia | DLBCL, NOS | IV | Concurrent | R-CHOP after 4 weeks of HLH regimen | ND | HLH-1994 regimen (4 weeks) | Death due to septic shock with multi-organ failure | 48 days |
| 8 | 24/M | Fever, splenomegaly, cytopenias, hemophagocytosis, hyperferritinemia, hypofibrinogemia, low NK activity, elevated IL-2R | EBV+, CD8+ peripheral T-cell lymphoma, NOS | IV | Concurrent | CHOP for four cycles + IT MTX (along with HLH-94 protocol), then brentuximab/etoposide for three cycles, followed by alemtuzumab for two cycles | RD | HLH-1994 regimen | Died under hospice care due to refractory disease | 279 days |
| 9 | 19/M | Fever, splenomegaly, cytopenias, hyperferritinemia, elevated IL-2R | EBV+, extranodal NK-/T-cell lymphoma | IAE | Concurrent | Carboplatin/etoposide for one cycle, followed by modified SMILE (w/o MTX) for one cycle, then carboplatin/gemcitabine/dexamethason/peg-asparaginase for four cycles (DDGP) | CR | Treatment of underlying malignancy | Alive in remission | 484 days as of last follow-up |
| Case | Age/sex | Race | Karyotype/FISH | IgH gene rearrangement | TCR gene rearrangement | EB virus in situ hybridization |
|---|---|---|---|---|---|---|
| ND: not done. | ||||||
| 1 | 53/F | Hispanic | 46XX | ND | ND | Negative |
| 2 | 51/F | Non-Hispanic (W) | 46XX | ND | ND | Negative |
| 3 | 54/M | Hispanic | 46XY | Positive | Negative | Positive |
| 4 | 55/F | Hispanic | 46XX | Negative | Negative | Positive |
| 5 | 70/F | Hispanic | BCL-6 rearrangement and t(3:14) | Positive | Negative | Negative |
| 6 | 52/M | Hispanic | 46,XY[20] | Negative | Negative | Positive |
| 7 | 73/F | Non-Hispanic (B) | 46,XX[20] | Positive | Negative | Negative |
| 8 | 24/M | Hispanic | 46, XY[20] | ND | Positive | Positive |
| 9 | 19/M | Hispanic | 46, XY[20] | ND | ND | Positive |