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2015, Number 5

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Gac Med Mex 2015; 151 (5)

Hyaline-vascular Multicentric Castleman’s Disease in an immunocompetent patient

Zapata-Bonilla SA, López-Vargas R, Scherling-Ocampo AA, Morales-Leyte AL, García-Ilizaliturri L
Full text How to cite this article

Language: Spanish
References: 18
Page: 648-654
PDF size: 976.71 Kb.


Key words:

Castleman’s disease, Hyaline-vascular, Lymphadenopathies, Crow-Fukake syndrome, Human herpes virus 8.

ABSTRACT

A previously healthy, immunocompetent 67-year-old female presented with a one-month history of general symptoms, weight loss, night fevers, and bilateral lower extremity edema. On admission she had severe anemia, acute kidney injury, and multiple lymphadenopathies. An excisional biopsy of one of the axillary lymphadenopathies confirmed hyaline-vascular Castleman’s disease. This rare disease is a polyclonal lymphoproliferative disorder that affects the normal lymph node architecture. According to its location it can be divided in unicentric (localized) or multicentric disease; it can be further divided according to histopathology in hyaline-vascular or plasmatic cells variety. Clinical presentation relates more to histopathological variety than to centricity. Human herpes virus 8 is ubiquitous in this disease and, along with interleukin 6, plays an important role in pathogenesis and symptoms presentation. Surgery is the go-to treatment of localized disease, while systemic chemotherapy is the option in multicentric disease. Communication between the clinical and anatomopathological teams is crucial; lag in diagnosis can lead to futile investigations in search of other diseases and delay in treatment.


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Gac Med Mex. 2015;151