Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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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


Table 1. Clinical Features and Outcomes
CaseAge/sexHLH criteriaAssociated malignancyStageInterval time between NHL and HLHNHL therapyNHL responseHLH treatmentOutcome/statusSurvival 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.
153/FFever, splenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, elevated sCD25Peripheral T-cell lymphoma, NOSIIHLH diagnosed at second lymphoma relapse, 36 months after initial lymphoma diagnosisCHOP, salvage ICE followed by autologous SCT, GEM/OX on 2° relapseCR to all three lines of therapyHLH-2004 regimen then allogeneic sibling SCTDied 13 days after allo-SCT due to massive cerebellar infarct298 days
251/FFever, splenomegaly, cytopenias, hemophagocytosis, hypertriglyceridemia, hyperferritinemiaPeripheral T-cell lymphoma, NOSPCNSLConcurrentCHOP-based regimen plus HD-MTXNDTreatment of underlying malignancyDied after cycle 1 of CHOP due to septic shock56 days
354/MFever, splenomegaly, cytopenias, hemophagocytosis , hypofibrinogenemia, hyperferritinemiaEBV+, extranodal NK-/T-cell lymphoma of the intestine (right colon, ileum)IEHLH diagnosed 11.9 weeks prior to NHLNARDHLH-1994 regimenDied in 4 months with refractory disease120 days
455/FFever, splenomegaly, cytopenias, hemophagocytosis , hypertriglyceridemia, hyperferritinemiaEBV+, PTLD-ALCL, ALK-IEHLH diagnosed 12.6 weeks after NHLTopical nitrogen mustardCRHLH-2004 regimenAlive in remission1,572 days as of last follow-up
570/FFever, splenomegaly, cytopenias, hemophagocytosis, hypertriglyceridemia, hyperferritinemiaDLBCL, NOSIVConcurrentVincristine/cyclophosphamide/methylprednisolone followed by R-CEOPRDTreatment of underlying malignancyDied under hospice care due to refractory disease50 days
652/MFever, splenomegaly, cytopenias, hyperferritinemia, hemophagocytosis, hypofibrinogemia, elevated IL-2REBV+, DLBCLIVBEConcurrentReduced dose cytoxan (five doses) + rituxan (two doses) followed by R-CHOEP + IT MTX for two cycles, then DA-REPOCH for four cyclesCRTreatment of underlying malignancyAlive in remission as of last encounter296 days as of last follow-up
773/FFever, splenomegaly, cytopenias, hemophagocytosis, hypertriglyceridemia, hyperferritinemiaDLBCL, NOSIVConcurrentR-CHOP after 4 weeks of HLH regimenNDHLH-1994 regimen (4 weeks)Death due to septic shock with multi-organ failure48 days
824/MFever, splenomegaly, cytopenias, hemophagocytosis, hyperferritinemia, hypofibrinogemia, low NK activity, elevated IL-2REBV+, CD8+ peripheral T-cell lymphoma, NOSIVConcurrentCHOP for four cycles + IT MTX (along with HLH-94 protocol), then brentuximab/etoposide for three cycles, followed by alemtuzumab for two cyclesRDHLH-1994 regimenDied under hospice care due to refractory disease279 days
919/MFever, splenomegaly, cytopenias, hyperferritinemia, elevated IL-2REBV+, extranodal NK-/T-cell lymphomaIAEConcurrentCarboplatin/etoposide for one cycle, followed by modified SMILE (w/o MTX) for one cycle, then carboplatin/gemcitabine/dexamethason/peg-asparaginase for four cycles (DDGP)CRTreatment of underlying malignancyAlive in remission484 days as of last follow-up


Table 2. Molecular Analysis
CaseAge/sexRaceKaryotype/FISHIgH gene rearrangementTCR gene rearrangementEB virus in situ hybridization
ND: not done.
251/FNon-Hispanic (W)46XXNDNDNegative
570/FHispanicBCL-6 rearrangement and t(3:14)PositiveNegativeNegative
773/FNon-Hispanic (B)46,XX[20]PositiveNegativeNegative
824/MHispanic46, XY[20]NDPositivePositive
919/MHispanic46, XY[20]NDNDPositive