Hepatosplenic T Cell Lymphoma Mimicking Hemophagocytic Lymphomhistiocytosis: A Case Report

Abstract Abstract 4864 Background. Hepatosplenic T cell lymphoma (HSTCL) is rare and aggressive extranodal lymphoma characteristically involves young males in third or fourth decade of life. It frequently manifests as pancytopenia with severe thrombocytopenia, hepatosplenomegaly, and much rarely, hemophagocytic lymphohistiocytosis (HLH). Methods. Medical records of a patient who presented with HLH as a presenting signs of HSTCL were reviewed. Literature search was performed to identify characteristic demographics and natural course of HSTCL reported to date. Results. A 30-year-old male presented after 6 months of constitutional symptoms. Remarkable findings were pancytopenia, mildly elevated LFT, and high ferritin level (89,000). Extensive work up for autoimmune and infectious etiology was negative. Worsening anemia and thrombocytopenia prompted the third bone marrow biopsy, which revealed the first evidence of hemophagocytosis. Despite of Cyclosporine A and Etoposide, pancytopenia worsened which prompted splenectomy and core needle liver biopsy. Sinusoids of spleen and liver were densely infiltrated with atypical lymphocytes consistent with T cell phenotype. Diagnosis of HSTCL was confirmed after PCR detection of gamma delta T cell receptor rearrangement. Previous bone marrow biopsies were retrospectively reviewed, which revealed small clusters of cells staining positive for CD3. The patient underwent three courses of chemotherapy that included high-dose Cytarabine, Etoposide and adriamycin. Post-chemotherapy course was complicated with disseminated Candidiasis complicated with mycortic aneurysm and worsening pancytopenia. The patient expired due to overwhelming septic shock 6 months after the pathologic diagnoses of HSTCL. Conclusion. HSTCL causes aberrant expansion of gamma delta T cells and defective innate immunity, and is an important secondary etiology for HLH. Splenectomy has diagnostic significance but little therapeutic benefit. Longer survival was reported in patients who underwent cytarabine-based chemotherapy; however median survivals in anecdotal case series all fall within 2 years regardless of regimen. Disclosures: No relevant conflicts of interest to declare.

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Intensive Induction Chemotherapy Followed by Early High-Dose Therapy and Hematopoietic Stem Cell Transplantation Results in Improved Outcome for Patients with Hepatosplenic T-Cell Lymphoma: A Single Institution Experience.

Blood ◽

10.1182/blood.v110.11.3454.3454 ◽

2007 ◽

Vol 110 (11) ◽

pp. 3454-3454

Author(s):

Martin Voss ◽

Andrew Zelenetz ◽

Esperanza B. Papadopoulos ◽

Hanna Weissbrot ◽

Steven M. Horwitz

Keyword(s):

Bone Marrow ◽

T Cell ◽

Cell Lymphoma ◽

Bone Marrow Involvement ◽

Prognostic Index ◽

T Cell Lymphoma ◽

High Dose ◽

Single Institution ◽

High Dose Therapy ◽

Hepatosplenic T Cell Lymphoma

Abstract Introduction: Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of non-Hodgkin lymphoma with unique features including presentation primarily in young men, lymphomatous infiltration of the liver and spleen, frequent bone marrow involvement, B symptoms, infrequent lymphadenopathy and poor prognosis. First described by Farcet and Gaulard1, there are two larger published series in whom only 6/66 of patients (pts) were alive at the time of the reports. 2,3 4/6 surviving pts in these reports had undergone high dose therapy and autologous or allogeneic stem cell transplantation (HDT-SCT). There are no prospective studies of treatment of HSTCL but a recent review of published case reports of HSTCL treated with allogeneic SCT suggests a better outcome for that approach.4 Methods: We reviewed our T-cell lymphoma and bone marrow transplantation databases to examine our results in pts with HSTCL. We identified 9 consecutive pts with this diagnosis. This report summarizes our single center experience. Results: All pts were male with a median age of 37y (12–59). All pts had stage IV disease with hepatomegaly and/or splenomegaly. 5/9 had documented bone marrow involvement, 7 had elevated LDH, and all 9 had B symptoms. Thrombocytopenia was present at diagnosis in 5 pts, anemia in 4 pts, and leukopenia in 4 pts. Transaminases and/or alkaline phosphatase were elevated in 6 pts. 4/9 had previous autoimmune disease: 2 with ulcerative colitis and 2 with rheumatoid arthritis. Responses to induction regimens were: CHOP (PR-2, POD-1) ICE/IVAC (CR-2, PR-2), pentostatin/2-CDA (POD-2). 2/4 pt achieved a CR to ICE as second line therapy. 8/9 pts achieved at least a PR and proceeded to HDT-SCT. 6 pts received an allogeneic SCT (one after relapse from autologous SCT), and 3 pts an autologous SCT. At the time of this report, 4/9 patients are alive in remission, 20–158 mos from diagnosis; the 4 surviving patients all underwent HDT/SCT. Following autologous-SCT 2/3 pts relapsed at 5 and 35 mos. Following Allogeneic-SCT 2/6 pts relapsed at 3 and 6 mos, 1 of whom was effectively treated with donor lymphocytes and remains in remission at 20 mos. 2/6 pts undergoing allo-SCT died of treatment related toxicities without documented recurrent disease. Complete information to determine the age-adjusted international prognostic index (aaIPI) was available for 8/9 pts; the aaIPI appeared to correlate with outcome: 4/5 pts with an aaIPI of low intermediate to high intermediate risk (1–2 factors) were alive compared to 0/3 aaIPI high risk disease (3 factors). The prognostic index for PTCL (PIT) consisting of age, performance status, LDH, and bone marrow involvement was also assessed. All 8 pts had at least one risk factor; 4/6 pts with a PIT of 1–2 were alive vs 0/2 pts for PIT of ≥3. Four pts received ICE or IVAC as their initial therapy and 3/4 were alive compared to only 1/5 for those who received other initial regimens. Conclusions: In this single institution experience, use of non-CHOP induction chemotherapy regimens such as ICE or IVAC and early use of HDT-SCT consolidation appear to improve the outcome for pts with HSTCL compared to reported results with CHOP or CHOP-like regimens.

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Hepatosplenic T Cell Lymphoma Case Report Presented with Hemphagocytosis Lymphohistiocytosis

Blood ◽

10.1182/blood-2019-126445 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 5363-5363

Author(s):

Mohamed Abu Haleeqa ◽

Fatemah Alshehi ◽

Gannat Alghamrawi ◽

Lois Richard

Keyword(s):

Bone Marrow ◽

Abdominal Pain ◽

T Cell ◽

Conflicts Of Interest ◽

Cell Lymphoma ◽

Multiorgan Failure ◽

Bone Marrow Aspiration ◽

T Cell Lymphoma ◽

Cell Phenotype ◽

Hepatosplenic T Cell Lymphoma

We are reporting a challenging case 37-year-old Pakistani male patient with Hepatosplenic T-Cell lymphoma (HSTCL) which is a rare aggressive form of Peripheral T-Cell lymphoma that is characterized by primary extra nodal disease with malignant T-cell proliferation in the liver, spleen, and bone marrow. He presented initially with nonspecific symptoms of generalized fatigue & vague abdominal pain . He was found to have hepatosplenomegaly (with liver & splenic lesions), derranged liver enzymes. He refused investigation and lost follow up. He came back 3 months after in a bad condition with fever, abdominal pain & he deteriorated very rapidly. The diagnosis was challenging as he required an extensive investigations that ultimately showed the characteristic clinical, histopathologic, and cytogenetic features of hepatosplenic T-cell lymphoma with HLH presentation. Patient had multiorgan failure,coagulopathy, shock(hepatic/renal/septic) and started on vasopressors, Ferritin was high >16000, Secondary Hemophagocytic Lymphohistiocytosis (HLH) was made at this point & patient was started on dexamethasone as per HLH protocol 2004 to reduce the tumor burden, and the plan was to start CHOP after 2 days, if patient condition allowed. Bone marrow biopsy showed: hypercellular Bone marrow massively infiltrated by neoplastic mature T-lineage population with overall morphologic & phenotypic characteristics consistent with T cell lymphoma. Flow cytometry was done from peripheral blood as bone marrow aspiration revealed dry tap.it showed presence of approximately 30-40% circulating lymphoma cells with aberrant mature T-cell phenotype, CD3+, TCRgd+, CD56+, CD8+/CD4-, and CD5-. The overall morphology and immunophenotypic features were consistent with hepatosplenic T-cell lymphoma (WHO classification updated 4th edition,2016). patient received full CHOP. All through patient was steadily worsening and unfortunately passed away. HSTCL is a rare and challenging diagnosis and presentation can be dramatic like this case with HLH. T cell lymphoma can present with HLH and treatment Figure Disclosures No relevant conflicts of interest to declare.

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