Journal of hematopathology · 2026
HIV-negative KSHV/HHV8-associated multicentric Castleman disease with concurrent Kaposi sarcoma and POEMS-like manifestations.
СтатьяКлинический случайНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Burul Gökhan, Tonyalı Büşra Tuğçe, Yalçınkaya İsa, Beyazyıldırım Sena, İnceman Merve, Serin İstemi
Аннотация
INTRODUCTION: Castleman disease (CD) is a heterogeneous lymphoproliferative disorder with unicentric and multicentric forms. Multicentric CD (MCD) is etiologically classified into idiopathic MCD (iMCD), POEMS-associated MCD, and Kaposi sarcoma-associated herpesvirus (KSHV/HHV-8)-associated MCD, and it typically presents with systemic inflammation. KSHV/HHV-8-associated MCD is most commonly seen in HIV-positive patients, although it may rarely occur in HIV-negative individuals.
CASE PRESENTATION: We report a case of a 65-year-old HIV-negative woman diagnosed with mixed-type KSHV/HHV-8-associated MCD accompanied by Kaposi sarcoma and POEMS-like clinical features. The patient presented with generalized lymphadenopathy, splenomegaly, cytopenias, hypercalcemia, and monoclonal IgG lambda gammopathy. PET-CT revealed widespread hypermetabolic lymphadenopathy and a sclerotic bone lesion in the left humerus. Bone marrow examination was normocellular and showed polytypic plasma cell proliferation. Lymph node biopsy demonstrated HHV-8 (LANA-1) positivity consistent with mixed-type MCD and Kaposi sarcoma. HHV-8 DNA positivity was also confirmed.
CONCLUSION: The patient was treated with a regimen including daratumumab, bortezomib, cyclophosphamide, and dexamethasone, achieving a clinical and hematologic response after the third cycle. This case highlights that KSHV/HHV-8-associated MCD may occur in HIV-negative individuals and may present concurrently with Kaposi sarcoma and POEMS-like manifestations, representing a rare diagnostic challenge.
CASE PRESENTATION: We report a case of a 65-year-old HIV-negative woman diagnosed with mixed-type KSHV/HHV-8-associated MCD accompanied by Kaposi sarcoma and POEMS-like clinical features. The patient presented with generalized lymphadenopathy, splenomegaly, cytopenias, hypercalcemia, and monoclonal IgG lambda gammopathy. PET-CT revealed widespread hypermetabolic lymphadenopathy and a sclerotic bone lesion in the left humerus. Bone marrow examination was normocellular and showed polytypic plasma cell proliferation. Lymph node biopsy demonstrated HHV-8 (LANA-1) positivity consistent with mixed-type MCD and Kaposi sarcoma. HHV-8 DNA positivity was also confirmed.
CONCLUSION: The patient was treated with a regimen including daratumumab, bortezomib, cyclophosphamide, and dexamethasone, achieving a clinical and hematologic response after the third cycle. This case highlights that KSHV/HHV-8-associated MCD may occur in HIV-negative individuals and may present concurrently with Kaposi sarcoma and POEMS-like manifestations, representing a rare diagnostic challenge.