TIM‐3 deficiency presenting with two clonally unrelated episodes of mesenteric and subcutaneous panniculitis‐like T‐cell lymphoma and hemophagocytic lymphohistiocytosis

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Abstract: This report offers novel clinical and diagnostic aspects of the association between germline mutations in HAVCR2 and subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL). The patient presented with panniculitis‐like T‐cell lymphoma involving mesenteric fatty tissue associated with hemophagocytic lymphohistiocytosis (HLH). Five years later, he developed a clonally unrelated SPTCL and underwent hematopoietic stem cell transplantation. Retrospectively, he was found to carry germline mutations in HAVCR2 associated with reduced T‐cell immunoglobulin mucin‐3 (TIM‐3) expression. We show that mesenteric fatty tissue localization of SPTCL can be the presenting manifestation of TIM‐3 deficiency, that this condition predisposes to recurrent lymphoma, and that flow cytometry is a possible screening tool

Location: Deutsche Nationalbibliothek Frankfurt am Main

Extent: Online-Ressource

Language: Englisch

Notes: Pediatric blood & cancer. - 67, 6 (2020) , e28302, ISSN: 1545-5017

Event: Veröffentlichung

(where): Freiburg

(who): Universität

(when): 2020

Creator: Wegehaupt, Oliver Philipp
Gross, Miriam
Wehr, Claudia
Marks, Reinhard
Schmitt-Graeff, Annette
Uhl, Markus
Lorenz, Myriam Ricarda
Schwarz, Klaus
Kratz, Christian Peter
Niemeyer, Charlotte
Ehl, Stephan

DOI: 10.1002/pbc.28302

URN: urn:nbn:de:bsz:25-freidok-1658993

Rights: Der Zugriff auf das Objekt ist unbeschränkt möglich.

Last update: 14.08.2025, 10:58 AM CEST

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2020

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TIM‐3 deficiency presenting with two clonally unrelated episodes of mesenteric and subcutaneous panniculitis‐like T‐cell lymphoma and hemophagocytic lymphohistiocytosis

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Other Objects (12)

Disease pathophysiology in murine models of primary hemophagocytic lymphohistiocytosis: triggers and effectors

Cell versus cytokine – directed therapies for hemophagocytic lymphohistiocytosis (HLH) in inborn errors of immunity

Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice

Adoptive T cell therapy cures mice from active hemophagocytic lymphohistiocytosis (HLH)

Adoptive T cell therapy cures mice from active hemophagocytic lymphohistiocytosis (HLH)

Immune‐mediated pathology as a consequence of impaired immune reactions: the IMPATH paradox

Trigger‐dependent differences determine therapeutic outcome in murine primary hemophagocytic lymphohistiocytosis

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Autoimmune lymphoproliferative immunodeficiencies (ALPID) in childhood: breakdown of immune homeostasis and immune dysregulation

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Disease pathophysiology in murine models of primary hemophagocytic lymphohistiocytosis: triggers and effectors

Cell versus cytokine – directed therapies for hemophagocytic lymphohistiocytosis (HLH) in inborn errors of immunity

Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice

Adoptive T cell therapy cures mice from active hemophagocytic lymphohistiocytosis (HLH)

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Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice

Adoptive T cell therapy cures mice from active hemophagocytic lymphohistiocytosis (HLH)

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