Malignant histiocytosis with a Langerhans cell subtype: A report on the diagnostic and therapeutic challenge

Abstract Hemophagocytosis (HP), a feature seen in malignant histiocytosis and infection- and lymphoma-associated disorders, has not been previously emphasized in Erdheim-Chester disease (ECD). Generally, ECD is recognized as a rare, systemic, non–Langerhans cell histiocytosis with a variable clinical course. Herein, we describe a unique case of multisystem non–Langerhans cell histiocytic proliferation with a fulminant clinical course (death occurred within 3 months of presentation) that showed prominent HP and extensive involvement of multiple organs, including the lungs, resulting in respiratory failure. Hemophagocytosis led to severe anemia that required transfusion and thrombocytopenia. Antemortem lung and bone marrow biopsy specimens revealed involvement by a histiocytic infiltrate with features highly suggestive of ECD and HP. Furthermore, the autopsy documented the presence of HP and the histiocytic infiltrate in multiple other organs. This case is best categorized as a variant form of ECD. Recognizing this variant has the following important implications: (1) HP may be a marker for fulminant clinical course in ECD, (2) the presence of HP does not exclude a diagnosis of ECD, and (3) ECD should be considered in the differential diagnosis of HP.

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Intracranial Tumorous Lesion as the First Manifestation of Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge - Case Report

Journal of Alzheimer’s Disease & Parkinsonism ◽

10.4172/2161-0460.1000298 ◽

2017 ◽

Vol 07 (01) ◽

Author(s):

Jolanta Florczak Wyspianska ◽

MichaAA Owecki ◽

MaAAgorzata Rzymkowska ◽

Halina Batura Gabryel ◽

Wojciech Kozubski

Keyword(s):

Case Report ◽

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Therapeutic Challenge ◽

Tumorous Lesion

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Langerhans cell tropism of human immunodeficiency virus type 1 subtype A through F isolates derived from different transmission groups.

Journal of Virology ◽

10.1128/jvi.71.10.8008-8013.1997 ◽

1997 ◽

Vol 71 (10) ◽

pp. 8008-8013 ◽

Cited By ~ 62

Author(s):

M T Dittmar ◽

G Simmons ◽

S Hibbitts ◽

M O'Hare ◽

S Louisirirotchanakul ◽

...

Keyword(s):

Human Immunodeficiency Virus ◽

Human Immunodeficiency Virus Type ◽

Virus Type ◽

Langerhans Cell ◽

Cell Tropism ◽

Immunodeficiency Virus ◽

Subtype A

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Comprehensive Immune Profiling of Lung Adenocarcinomas Reveals Four Immunosubtypes with Plasma Cell Subtype a Negative Indicator

Cancer Immunology Research ◽

10.1158/2326-6066.cir-15-0214 ◽

2016 ◽

Vol 4 (3) ◽

pp. 234-247 ◽

Cited By ~ 32

Author(s):

Yutaka Kurebayashi ◽

Katsura Emoto ◽

Yuichiro Hayashi ◽

Ikuo Kamiyama ◽

Takashi Ohtsuka ◽

...

Keyword(s):

Plasma Cell ◽

Cell Subtype ◽

Subtype A ◽

Lung Adenocarcinomas ◽

Immune Profiling

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Thyroid involvement by malignant histiocytosis of Langerhans' cell type

Clinical Endocrinology ◽

10.1046/j.1365-2265.1996.8020819.x ◽

1996 ◽

Vol 45 (3) ◽

pp. 357-363 ◽

Cited By ~ 24

Author(s):

Shinji Kitahama ◽

Makoto Iitaka ◽

Tadashi Shimizu ◽

Naoki Serizawa ◽

Nobuhiko Fukasawa ◽

...

Keyword(s):

Langerhans Cell ◽

Cell Type ◽

Malignant Histiocytosis

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Prolymphocytic leukaemia of B- and T-cell subtype: a state-of-the-art paper

European Journal Of Haematology ◽

10.1111/j.1600-0609.2008.01069.x ◽

2008 ◽

Vol 80 (6) ◽

pp. 469-476 ◽

Cited By ~ 54

Author(s):

M. Dungarwalla ◽

E. Matutes ◽

C. E. Dearden

Keyword(s):

T Cell ◽

State Of The Art ◽

Cell Subtype ◽

Prolymphocytic Leukaemia ◽

Subtype A

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A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge

Case Reports in Hematology ◽

10.1155/2018/7865325 ◽

2018 ◽

Vol 2018 ◽

pp. 1-6 ◽

Cited By ~ 1

Author(s):

Hamza Hashmi ◽

Drew Murray ◽

John Greenwell ◽

Marwan Shaikh ◽

Soumit Basu ◽

...

Keyword(s):

Interferon Alpha ◽

Langerhans Cell Histiocytosis ◽

Rare Case ◽

Langerhans Cell ◽

Braf V600e ◽

Therapeutic Challenge ◽

Erdheim Chester Disease ◽

Organ Systems ◽

Erdheim Chester ◽

Chester Disease

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocyte disorder most commonly characterized by multifocal osteosclerotic lesions of the long bones demonstrating sheets of foamy histiocyte infiltrates on biopsy with or without histiocytic infiltration of extraskeletal tissues. ECD can be difficult to diagnose since it is a very rare disease that can affect many organ systems. Diagnosis is based on the pathologic evaluation of involved tissue interpreted within the clinical context. Patients who have the BRAF V600E mutation are treated first line with vemurafenib. For those without the mutation with symptomatic ECD, conventional or PEGylated interferon alpha is recommended. For patients who are either intolerant or nonresponsive to interferon alpha, systemic chemotherapy with or without corticosteroids can be used. We present a rare case of Erdheim-Chester disease with concurrent Langerhans cell histiocytosis which occurs in only one fifth of the cases and often presents as a diagnostic and therapeutic challenge.

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