Hepatosplenic T-cell lymphoma: Report of two cases

Hepatosplenic T-cell lymphoma (HSTCL) is a rare type of lymphoma. Presenting with hepatosplenomegaly, fever, cytopenia without significant lymphadenopathy, most patients are young men with poor outcomes. Here is the report of two cases of HSTCL from Maharaj Nakorn Chiang-Mai Hospital, Thailand. Both patients were middle-aged men presented with prolonged fever, hepatosplenomegaly and cytopenia. Abnormal lymphoid cells, not demonstrated by flow cytometry, were microscopically revealed in the patients’ bone marrow. The diagnosis of HSTCL was based on the histopathologic section obtained from splenectomy and liver biopsy. Both patients received cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) regimen initially while one of them was salvaged with etoposide, methylprednisolone, cytarabine, cisplatin (ESHAP) and methotrexate, high-dose cytarabine, methylprednisolone (cycle B of hyperCVAD) regimen. They responded poorly to chemotherapy and succumbed to severe sepsis. The presented cases confirmed that HSTCL is very difficult to diagnose and current treatment modalities appear to be ineffective

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Hepatosplenic T Cell Lymphoma Mimicking Hemophagocytic Lymphomhistiocytosis: A Case Report

Blood ◽

10.1182/blood.v120.21.4864.4864 ◽

2012 ◽

Vol 120 (21) ◽

pp. 4864-4864

Author(s):

Inhye E Ahn ◽

Lawrence Rice

Keyword(s):

Bone Marrow ◽

T Cell ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Cell Phenotype ◽

High Dose ◽

Severe Thrombocytopenia ◽

Gamma Delta ◽

Bone Marrow Biopsies ◽

Hepatosplenic T Cell Lymphoma

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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A comparison of treatment modalities for nasal extranodal natural killer/T-cell lymphoma in early stages: The efficacy of CHOP regimen based concurrent chemoradiotherapy

Oncotarget ◽

10.18632/oncotarget.13614 ◽

2016 ◽

Vol 8 (12) ◽

pp. 20362-20370 ◽

Cited By ~ 4

Author(s):

Jianzhong Cao ◽

Shengmin Lan ◽

Liuhai Shen ◽

Hongwei Si ◽

Ning Zhang ◽

...

Keyword(s):

T Cell ◽

Natural Killer ◽

Concurrent Chemoradiotherapy ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Treatment Modalities ◽

Chop Regimen ◽

Natural Killer T Cell ◽

Early Stages ◽

Killer T Cell

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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 with hepatocytotropism in a cat

Journal of Feline Medicine and Surgery Open Reports ◽

10.1177/20551169211005914 ◽

2021 ◽

Vol 7 (1) ◽

pp. 205511692110059

Author(s):

Tatsuhito Ii ◽

James K Chambers ◽

Kazuhito Segawa ◽

Kazuyuki Uchida

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

Granzyme B ◽

Lymphoid Cells ◽

T Cell Lymphoma ◽

Cytological Examination ◽

Hepatic Sinusoid ◽

Case Summary ◽

Mixed Breed ◽

Hepatosplenic T Cell Lymphoma

Case summary A 14-year 3-month-old spayed female mixed-breed cat presented with jaundice, anaemia and thrombocytopenia. Haemophagocytic syndrome associated with lymphoma was suspected after cytological examination of the spleen. Despite treatment with prednisolone, L-asparaginase and nimustine, the cat died 176 days after the initial presentation. Necropsy revealed splenomegaly and hepatomegaly, without lymphadenopathy. Histopathologically, neoplastic lymphoid cells infiltrated the hepatic sinusoid and splenic sinus. The neoplastic lymphoid cells showed marked hepatocytotropism and contained erythrocytes, which was also confirmed by electron microscopy. Immunohistochemically, neoplastic lymphoid cells were positive for CD3, TIA1 (GMP-17) and granzyme B, and negative for CD8, CD20, CD56, CD57, CD79a and Iba1. Based on these findings, the cat was diagnosed with hepatosplenic T-cell lymphoma (HS-TCL) with hepatocytotropism. Relevance and novel information This case shows cytotoxic immunophenotype of HS-TCL in a cat, which has not been demonstrated before. Severe hepatocytotropism and haemophagocytosis of the neoplastic cells were likely to be associated with jaundice and anaemia, respectively, and the poor outcome of the present case.

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Inert CD30-positive extranodal NK/T cell lymphoma with large cell transformation: a case report

10.21203/rs.3.rs-16556/v1 ◽

2020 ◽

Author(s):

Hui Wang ◽

PengYi Yu ◽

Qing Li

Keyword(s):

T Cell ◽

Cell Transformation ◽

Cell Lymphoma ◽

Large Cell ◽

Lymphoid Cells ◽

T Cell Lymphoma ◽

Epstein Barr Virus Infection ◽

Rare Type ◽

Large Cell Transformation ◽

Nk T Cell

Abstract Background Extranodal natural killer (NK)/T-Cell Lymphoma is a rare type of cytotoxic lymphoma associated with Epstein-Barr virus infection, highly invasive and relatively resistant to chemotherapy. Inert CD30-positive extranodal NK/T cell lymphoma with large cell transformation is very rare. Case presentation A married 55-year-old male was admitted to hospital in February 2017, because of “diagnosis of malignant lymphoma for about 12 years with high fever and a left neck mass present for about 3 weeks". The patient was diagnosed to NK/T Cell Lymphoma as early as 2006, repeated relapses in June 2010 and April 2013. The patient was biopsied again in February 2017, lymph node, about 2.5×2×1.3cm.The section was off-white and tender. Microscopic examinations showed a diffuse proliferation of small and atypical lymphoid cells, admixed with large lymphoid cells. Immunohistochemically, the tumor cells exhibited positivity for CD30, CD3, CD43, CD2, CD56, TIA, negativity for CD4, CD8, AE1/AE3, CD20, ALK, EMA staining. In situ hybridization for EBER was strongly positive in the specimens.Conclusions Herein, we report a case of CD30-positive extranodal NK/T Cell Lymphoma with large cell transformation, nasal type, in indolent course over a period of 12 years. The accurate diagnosis of NK/T-cell lymphoma with CD30 expression and large cell transformation is very important. The disease usually has a poor prognosis related to several factors, but the indolent behavior of the present case is more unusual. A long-term follow-up is suggested to be performed to inspect the progression for this tumor.

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