Mendoza Romo-Ramírez MÁ, Alejo-Fuentes LF, Stahl-López GA, Pérez-Romero AG

Language: Spanish
References: 13
Page: 184-189
PDF size: 309.11 Kb.

ABSTRACT

Background: Castleman disease is a rare disease belonging to a heterogeneous group of lymphoproliferative disorders, first described by Dr. Benjamin Castle in the decade of 1950. It has two distinct clinical presentations: the subtypes unicentric (UCD) and the multicentric (MCD), with more aggressive node involvement. The multicentric Castleman disease form acts like lymphoma without being a cancer, and eventually a group of these patients develop lymphoma and are treated accordingly. The median age of presentation is in young adults, with discrete female predominance. The diagnosis is made through clinical findings and a histopathological pattern. The unicentric Castleman disease is the hyaline vascular variant found in 80% of cases. The MCD form is the plasmatic cell variant with the 20% of the cases. Unicentric Castleman disease can be cured by surgically removing the node, whereas the treatment options against the multicentric Castleman disease may include corticosteroids, immunotherapy (rituximab), radiotherapy or AVBD chemotherapy.
Clinical case: A 49-year-old male patient presenting malaise, several enlarged lymph nodes and B symptoms, altogether along with histological findings of hyaline vascular variant integrate the unicentric Castleman disease subtype.
Conclusions: Castleman’s disease can be considered a benign disease with a good prognosis depending on the clinical and histopathological variant, since it is not exempt from progression.

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