Hemophagocytic Lymphohistiocytosis Secondary to T Cell/Histiocyte-rich Large B Cell Lymphoma in an Adolescent Male

Hemophagocytic Lymphohistiocytosis (HLH) is a hyperinflammatory disorder that may be encountered as a primary or secondary phenomenon. HLH secondary to lymphoma has been described, more frequently in adults than in children. T-cell/Histiocyte-rich B-cell lymphoma (THRLBCL) is a large B-cell lymphoma that resides in a microenvironment of robust host immune response and has previously been associated with HLH in adults. Here, we describe the first case of HLH secondary to THRLBCL in an adolescent patient.

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Hemophagocytic lymphohistiocytosis secondary to T-cell/histiocyte-rich large B-cell lymphoma

Leukemia Research Reports ◽

10.1016/j.lrr.2014.05.004 ◽

2014 ◽

Vol 3 (2) ◽

pp. 42-45 ◽

Cited By ~ 4

Author(s):

Katherine Devitt ◽

Jan Cerny ◽

Bradley Switzer ◽

Muthalagu Ramanathan ◽

Rajneesh Nath ◽

...

Keyword(s):

T Cell ◽

B Cell ◽

Hemophagocytic Lymphohistiocytosis ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

Large B Cell

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T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

Case Reports in Oncological Medicine ◽

10.1155/2017/6428461 ◽

2017 ◽

Vol 2017 ◽

pp. 1-5

Author(s):

Uroosa Ibrahim ◽

Gwenalyn Garcia ◽

Amina Saqib ◽

Shafinaz Hussein ◽

Qun Dai

Keyword(s):

T Cell ◽

B Cells ◽

B Cell ◽

Rare Disease ◽

Hemophagocytic Lymphohistiocytosis ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Biologically Active ◽

Large B Cell Lymphoma ◽

Large B Cell

T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkin’s lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy. On presentation, he had a fever of 105°F. Laboratory work-up was consistent with pancytopenia, elevated lactate dehydrogenase, elevated D-dimer, and a ferritin of 24,247 ng/mL. The patient was started on steroid therapy. An excisional biopsy of the right inguinal lymph node was consistent with a diagnosis of THRLBCL and the patient subsequently received six cycles of chemotherapy with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) after which a PET-CT scan showed no evidence of biologically active disease and ferritin was down to 822 ng/mL. We discuss the clinical manifestations and diagnostic and therapeutic considerations of this rare disease along with a review of reported cases in the literature.

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A Case of Spontaneously Improving Secondary Hemophagocytic Lymphohistiocytosis in an Adult Associated with T-Cell Histiocyte-Rich Large B-Cell Lymphoma

Case Reports in Oncological Medicine ◽

10.1155/2018/8069182 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Cyrus Askin ◽

Ashley Burris ◽

Clifton Layman ◽

Brian Haney ◽

Jordan Hall

Keyword(s):

T Cell ◽

B Cell ◽

Hemophagocytic Lymphohistiocytosis ◽

Nk Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Documented Case ◽

Large B Cell Lymphoma ◽

Large B Cell ◽

Secondary Hemophagocytic Lymphohistiocytosis

Secondary hemophagocytic lymphohistiocytosis (HLH) in adults is a rare, often fatal syndrome characterized by widespread immune dysregulation. It is seen as a complication of infections, autoimmune diseases, and malignancies. Among the malignancy-related causes, aggressive T-cell or NK-cell neoplasms are most notable, while B-cell lymphomas are less commonly implicated. We present the case of a 32-year-old male transferred to our facility with concern for HLH. During the first week of his hospitalization, his diagnosis was confirmed and the patient demonstrated spontaneous improvement in his symptoms prompting us to delay therapy while searching for a primary cause. In the second week, the patient deteriorated, leading us to initiate steroid monotherapy in the absence of a cause for his HLH. Meanwhile, pathology results from an excisional lymph node biopsy confirmed a diagnosis of T-cell/histiocyte-rich large B-cell lymphoma (TCHRLBCL). Subsequently, we initiated therapy with dose-adjusted R-EPOCH. The patient achieved a complete remission of both HLH and TCHRLBCL as well as a complete return to his prior functional status. In our review of the literature, this represents only the second documented case of HLH associated with TCHRLBCL and the only documented case of an adult experiencing significant spontaneous recovery in this context.

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T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: Report of the First Case in the Mandible

Head and Neck Pathology ◽

10.1007/s12105-018-0948-9 ◽

2018 ◽

Vol 13 (4) ◽

pp. 711-717

Author(s):

Ricardo Natã Fonseca Silva ◽

Elismauro Francisco Mendonça ◽

Aline Carvalho Batista ◽

Rita de Cássia Gonçalves Alencar ◽

Ricardo Alves Mesquita ◽

...

Keyword(s):

T Cell ◽

B Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

First Case ◽

Large B Cell

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T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Masquerading as Autoimmune Hepatitis with Clinical Features of Hemophagocytic Lymphohistiocytosis

Journal of Gastrointestinal & Digestive System ◽

10.4172/2161-069x.1000283 ◽

2015 ◽

Vol 05 (03) ◽

Author(s):

Minesh Mehta, Jiang

Keyword(s):

T Cell ◽

B Cell ◽

Autoimmune Hepatitis ◽

Clinical Features ◽

Hemophagocytic Lymphohistiocytosis ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

Large B Cell

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First case of T-cell/histiocyte-rich-large B-cell lymphoma presenting with duodenal obstruction

Libyan Journal of Medicine ◽

10.3402/ljm.v8i0.22955 ◽

2013 ◽

Vol 8 (1) ◽

pp. 22955 ◽

Cited By ~ 1

Keyword(s):

T Cell ◽

B Cell ◽

Duodenal Obstruction ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

First Case ◽

Large B Cell

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Use of immune repertoire sequencing to resolve discordant microscopic and immunochemical findings in a case of T cell-rich large B cell lymphoma in a young dog

BMC Veterinary Research ◽

10.1186/s12917-021-02783-3 ◽

2021 ◽

Vol 17 (1) ◽

Author(s):

Gary Kwok Cheong Lee ◽

Dorothee Bienzle ◽

Stefan Matthias Keller ◽

Mei-Hua Hwang ◽

Nikos Darzentas ◽

...

Keyword(s):

T Cells ◽

T Cell ◽

B Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Immune Repertoire ◽

Large B Cell Lymphoma ◽

Repertoire Sequencing ◽

Reactive Lymphocytes ◽

Large B Cell

Abstract Background Lymphocytic neoplasms with frequent reactive lymphocytes are uncommonly reported in dogs, and can pose a diagnostic challenge. Different diagnostic modalities such as cytology, flow cytometry, histopathology, immunohistochemistry, and clonality testing, are sometimes required for a diagnosis. This report illustrates the value of using a multi-modal diagnostic approach to decipher a complex lymphocytic tumor, and introduces immune repertoire sequencing as a diagnostic adjunct. Case presentation A 10-month-old Great Dane was referred for marked ascites. Cytologic analysis of abdominal fluid and hepatic aspirates revealed a mixed lymphocyte population including numerous large lymphocytes, yielding a diagnosis of lymphoma. Flow cytometrically, abdominal fluid lymphocytes were highly positive for CD4, CD5, CD18, CD45, and MHC II, consistent with T cell lymphoma. Due to a rapidly deteriorating clinical condition, the dog was euthanized. Post mortem histologic evaluation showed effacement of the liver by aggregates of B cells surrounded by T cells, suggestive of hepatic T cell-rich large B cell lymphoma. Immune repertoire sequencing confirmed the presence of clonal B cells in the liver but not the abdominal fluid, whereas reactive T cells with shared, polyclonal immune repertoires were found in both locations. Conclusions T cell-rich large B cell lymphoma is a rare neoplasm in dogs that may be challenging to diagnose and classify due to mixed lymphocyte populations. In this case, the results of histopathology, immunohistochemistry and immune repertoire sequencing were most consistent with a hepatic B cell neoplasm and reactive T cells exfoliating into the abdominal fluid. Immune repertoire sequencing was helpful in delineating neoplastic from reactive lymphocytes and characterizing repertoire overlap in both compartments. The potential pitfalls of equating atypical cytomorphology and monotypic marker expression in neoplasia are highlighted.

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