Case Report: Large Granular Lymphocyte Leukemia (LGLL)—A Case Series of Challenging Presentations

Medientyp: E-Artikel

Titel: Case Report: Large Granular Lymphocyte Leukemia (LGLL)—A Case Series of Challenging Presentations

Beteiligte: Pflug, Natali [Verfasser:in]; Littauer, Annika [Verfasser:in]; Beverungen, David [Verfasser:in]; Sretenovic, Aleksandra [Verfasser:in]; Wahnschaffe, Linus [Verfasser:in]; Braun, Till [Verfasser:in]; Dechow, Annika [Verfasser:in]; Jungherz, Dennis [Verfasser:in]; Otte, Moritz [Verfasser:in]; Monecke, Astrid [Verfasser:in]; Bach, Enrica [Verfasser:in]; Franke, Georg-Nikolaus [Verfasser:in]; Schwind, Sebastian [Verfasser:in]; Jentzsch, Madlen [Verfasser:in]; Platzbecker, Uwe [Verfasser:in]; Herling, Marco [Verfasser:in]; Vucinic, Vladan [Verfasser:in]

Erschienen: Lausanne: Frontiers Research Foundation, [2023]

Sprache: Englisch

Schlagwörter: TCR ; LGL leukemia ; CLPD-NK ; NK ; immunosuppression ; STAT-3

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Beschreibung: Large granular lymphocyte leukemia (LGLL) represents a rare group of diseases withconsiderable difficulties in their correct diagnostic workup and therapy. The majorchallenges lie in their distinction from reactive (including autoimmune)lymphoproliferations. Moreover, monoclonal LGL proliferative diseases are in fact aheterogeneous group of disorders, as recognized by the three subtypes in the currentWHO classification. It distinguishes two chronic forms (the focus of this case series),namely T-LGLL and chronic lymphoproliferative disorders of Natural Killer cells (CLPD-NK)as well as aggressive NK-cell leukemia. In the clinical routine, the variable presentationsand phenotypes of T-LGLL and CLPD-NK are underappreciated. The relevant differentialdiagnoses range from benign reactive T-cell expansions to other mature T-cell leukemiasto highly aggressive gd-lymphomas. T-LGLL or CLPD-NK patients suffer from a widevariety of symptoms often including, but not limited to, cytopenias or classicalautoimmune phenomena. They receive treatments ranging from mere supportivemeasures (e.g. antibiotics, growth factors, transfusions) over strategies ofimmunosuppression up to anti-leukemic therapies. The diagnostic pitfalls range fromrecognition of the subtle T-cell proliferation, repeated establishment of monoclonality,assignment to a descript immunophenotypic pattern, and interpretations of molecularaberrancies. Here, we report a series of selected cases to represent the spectrum ofLGLL. The purpose is to raise awareness among the scientifically or practically interestedreaders of the wide variety of clinical, immunological, and phenotypic features of thevarious forms of LGLL, e.g. of T-cell type, including its gd forms or those of NK-lineage. Wehighlight the characteristics and courses of four unique cases from two academic centers,including those from a prospective nationwide LGLL registry. Each case of this instructivecatalogue serves to transport a key message from the areas of (chronic inflammatory) contexts in which LGLL can arise as well as from the fields of differential diagnostics and ofvarious treatment options. Implications for optimization in these areas are discussed.

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Rechte-/Nutzungshinweise: Namensnennung (CC BY)

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