scholarly journals Multicentric Castleman disease in a DOCK8‐deficient patient with Orf virus infection

2021 ◽  
Author(s):  
Elif Tugce Aydin Goker ◽  
Deniz Cagdas ◽  
Inci Yaman Bajin ◽  
Musa Gurel Kukul ◽  
Elif Soyak Aytekin ◽  
...  
Keyword(s):  
Virus Infection ◽  
Castleman Disease ◽  
Orf Virus ◽  
Multicentric Castleman Disease ◽  
Deficient Patient

scholarly journals Multicentric Castleman Disease in a DOCK8 Deficient Patient with Orf Virus Infection

2021 ◽  
Author(s):  
Elif Tugce Aydin Goker ◽  
Deniz Cagdas Ayvaz ◽  
Inci Bajin ◽  
Musa Kukul ◽  
Elif Soyak Aytekin ◽  
...  
Keyword(s):  
Herpes Virus ◽  
Castleman Disease ◽  
Orf Virus ◽  
Human Herpes ◽  
Human Herpes Virus 8 ◽  
Multicentric Castleman Disease ◽  
Human Herpes Virus ◽  
Dock8 Deficiency ◽  
Deficient Patient ◽  
Herpès Virus

Castleman disease is a rare, heterogeneous disorder that driven by proinflammatory responses. Human herpes virus-8 has a major role in pathogenesis of multicentric Castleman disease. There is also a subgroup of cases, human herpes virus-8 negative, idiopathic multicentric Castleman disease. The role of primary immunodeficiencies in idiopathic Castleman disease are poorly described. DOCK8 deficiency is a combined primary immunodeficiency. It has a broad clinic spectrum including atopy, autoimmunity and cancer. We present a 10-year-old, DOCK8 deficient patient. He had giant lobular capillary hemangiomas on his neck, iliac and gluteal regions and multiple lymphadenopathies. Abdominal lymph node pathology revealed hyaline vascular type Castleman disease and human herpes virus-8 staining was negative. His lesions were shown to be infected with orf virus. Our case is the first case to relate idiopathic multicentric Castleman disease and DOCK8 deficiency; also, very unusual presentation of orf virus infection in humans.

scholarly journals “Chronic Disseminated Aspergillosis,” a Novel Fungal Immune Reconstitution Inflammatory Syndrome

2020 ◽  
Vol 7 (11) ◽  
Author(s):  
Annabelle Pourbaix ◽  
Romain Guery ◽  
Julie Bruneau ◽  
Estelle Blanc ◽  
Gregory Jouvion ◽  
...  
Keyword(s):  
Immune Reconstitution Inflammatory Syndrome ◽  
Immune Reconstitution ◽  
Clinical Presentation ◽  
Castleman Disease ◽  
Disease Symptoms ◽  
Disseminated Candidiasis ◽  
Multicentric Castleman Disease ◽  
Chronic Disseminated Candidiasis ◽  
Disseminated Aspergillosis ◽  
Inflammatory Syndrome

Abstract We report a case of chronic hepatosplenic aspergillosis following immune reconstitution complicating colic aspergillosis in an AIDS patient with multicentric Castleman disease. Symptoms mimicked the clinical presentation of chronic disseminated candidiasis and responded to corticosteroid. This emerging entity enlarges the spectrum of fungal immune reconstitution inflammatory syndrome in the HIV setting.

Validated International Definition of the TAFRO clinical subtype of idiopathic multicentric Castleman disease

2021 ◽  
Author(s):  
Yoshito Nishimura ◽  
David C. Fajgenbaum ◽  
Sheila K. Pierson ◽  
Noriko Iwaki ◽  
Asami Nishikori ◽  
...  
Keyword(s):  
Castleman Disease ◽  
Multicentric Castleman Disease ◽  
Definition Of ◽  
Clinical Subtype

Coexistence of disseminated Kaposi sarcoma and multicentric Castleman disease in an HIV-infected patient under viral suppression

2021 ◽  
Vol 32 (3) ◽  
pp. 286-289
Author(s):  
I-Fan Lin ◽  
Jiun-Nong Lin ◽  
Tsung-Heng Tsai ◽  
Chao-Tien Hsu ◽  
Yu-Ying Wu ◽  
...  
Keyword(s):  
Viral Load ◽  
Viral Suppression ◽  
Liposomal Doxorubicin ◽  
Kaposi Sarcoma ◽  
Castleman Disease ◽  
Severe Thrombocytopenia ◽  
Multicentric Castleman Disease ◽  
Progressive Pancytopenia ◽  
One Year ◽  
Cd4 T Cell Count

Coexistence of multicentric Castleman disease and Kaposi sarcoma is rare and might be missed without an experienced pathologists’ interpretation. A 46-year-old man had been diagnosed with HIV infection and treated with combination antiretroviral therapy of dolutegravir/abacavir/lamivudine (Triumeq) for one year. The latest viral load was 49 copies/mL and CD4 T-cell count was 192 cells/uL. He was admitted due to fever off and on, splenomegaly, general lymphadenopathy, and severe thrombocytopenia for two months. Biopsy of a purplish skin lesion and gastric tissue showed Kaposi sarcoma. The pathology of inguinal lymph nodes revealed coexistence of Kaposi sarcoma and multicentric Castleman disease. The plasma Kaposi sarcoma herpesvirus viral load was 365,000 copies/mL. During hospitalization, progressive pancytopenia and spiking fever persisted, and he died of multi-organ failure before completion of chemotherapeutic treatments with rituximab plus liposomal doxorubicin.

scholarly journals A Review of Genetic Abnormalities in Unicentric and Multicentric Castleman Disease

Biology ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 251
Author(s):  
Alexandra Butzmann ◽  
Jyoti Kumar ◽  
Kaushik Sridhar ◽  
Sumanth Gollapudi ◽  
Robert S. Ohgami
Keyword(s):  
Plasma Cells ◽  
Treatment Options ◽  
Disease Process ◽  
Castleman Disease ◽  
Mitogen Activated Protein Kinase ◽  
Human Herpes Virus 8 ◽  
Multicentric Castleman Disease ◽  
Molecular Abnormalities ◽  
Genetic Abnormalities ◽  
Mitogen Activated Protein

Castleman disease (CD) is a rare lymphoproliferative disorder known to represent at least four distinct clinicopathologic subtypes. Large advancements in our clinical and histopathologic description of these diverse diseases have been made, resulting in subtyping based on number of enlarged lymph nodes (unicentric versus multicentric), according to viral infection by human herpes virus 8 (HHV-8) and human immunodeficiency virus (HIV), and with relation to clonal plasma cells (POEMS). In recent years, significant molecular and genetic abnormalities associated with CD have been described. However, we continue to lack a foundational understanding of the biological mechanisms driving this disease process. Here, we review all cases of CD with molecular abnormalities described in the literature to date, and correlate cytogenetic, molecular, and genetic abnormalities with disease subtypes and phenotypes. Our review notes complex karyotypes in subsets of cases, specific mutations in PDGFRB N666S in 10% of unicentric CD (UCD) and NCOA4 L261F in 23% of idiopathic multicentric CD (iMCD) cases. Genes affecting chromatin organization and abnormalities in methylation are seen more commonly in iMCD while abnormalities within the mitogen-activated protein kinase (MAPK) and interleukin signaling pathways are more frequent in UCD. Interestingly, there is a paucity of genetic studies evaluating HHV-8 positive multicentric CD (HHV-8+ MCD) and POEMS-associated CD. Our comprehensive review of genetic and molecular abnormalities in CD identifies subtype-specific and novel pathways which may allow for more targeted treatment options and unique biologic therapies.

scholarly journals HHV8-Positive Castleman Disease and In Situ Mantle Cell Neoplasia within Dermatopathic Lymphadenitis, in Longstanding Psoriasis

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1150
Author(s):  
Magda Zanelli ◽  
Luca Stingeni ◽  
Maurizio Zizzo ◽  
Giovanni Martino ◽  
Francesca Sanguedolce ◽  
...  
Keyword(s):  
Mantle Cell Lymphoma ◽  
Cell Lymphoma ◽  
Rare Event ◽  
Castleman Disease ◽  
Axillary Lymph ◽  
Tnf Inhibitor ◽  
Dermatopathic Lymphadenitis ◽  
Multicentric Castleman Disease ◽  
Mantle Cell

A 73-year-old man presented with multiple lymphadenopathy. He had a 20-year history of palmoplantar psoriasis evolved to a diffuse erythrodermic picture in the last two years. Topic and systemic medications including prednisolone, acitretin, anti-IL17 (ixekizumab), TNF inhibitor (adalimumab), anti-IL23 (guselkumab), methotrexate, cyclosporine, and phosphodiesterase 4 inhibitor (apremilast) were ineffective. Repeated skin biopsies excluded mycosis fungoides, confirming psoriasis; molecular analysis of T-cell receptor genes ruled out clonality. The axillary lymph node histology documented a dermatopathic lymphadenitis, often associated with chronic cutaneous inflammatory diseases. At an accurate morphological evaluation, features of HHV8-positive multicentric Castleman disease were observed. Moreover, in a few follicles, in situ mantle cell neoplasia was identified. The translocation t(11;14)(q13;q32), characteristic of mantle cell lymphoma, and the monoclonal IGH gene rearrangement were present. HHV8 DNA was identified on plasma sample. Multicentric Castleman disease in psoriatic patients is a rare event and it might be favored by the immunomodulatory treatment in longstanding psoriasis. Multicentric Castleman disease patients are predisposed to developing simultaneous or subsequent lymphoma. In situ mantle cell neoplasia often behaves indolently, although it may progress to overt mantle cell lymphoma. Rituximab achieved a good control of psoriasis. Unfortunately, the patient developed Staphylococcus aureus sepsis for which he is currently on antibiotic therapy.

scholarly journals Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease

2016 ◽  
Vol 91 (2) ◽  
pp. 220-226 ◽  
Author(s):  
Noriko Iwaki ◽  
David C. Fajgenbaum ◽  
Christopher S. Nabel ◽  
Yuka Gion ◽  
Eisei Kondo ◽  
...  
Keyword(s):  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease ◽  
Distinct Subtype
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