Multicentric Castleman Disease a Diagnostic Dilemma: A Case Series of Three Patients Seen at a Tertiary Health Facility in Zambia

Abstract

Background: Castleman disease (CD) is a heterogeneous group of lymphoproliferative disorders of uncertain cause presenting with lymphadenopathy. There are three generally described immunological disorders of CD that occur in individuals of all ages and share a similar microscopic lymph node appearance but different immunohistochemical profile. Oftentimes, diagnosis can be challenging where histology is not done, with most patients being presumptively treated as tuberculous (TB) adenitis.

Method & Results: We present three case reports of CD, one is of an Human Immunodeficiency Virus (HIV) naive patient, the other two are in HIV positive patients. All three patients presented with fever, recurrent anemia and generalized lymphadenopathy. W hole body Computed Tomography (CT) scans as well as excision biopsies (for histopathology and immunohistochemical staining for Human Herpes Virus-8 (HHV-8) were done on all three patients.

Case 1: Idiopathic Multicentric Castleman Disease in a 20 year old male patient who also developed TAFRO syndrome (Thrombocytopenia, Ascites, myelo Fibrosis, Renaldys functionand Organomegaly) in the course of the illness. Case 2 & 3: HHV-8 associated Multi centric Castleman Disease in HIV positive 42 year and 27 year old female patients respectively. The 42 year old received chemotherapy for six months, and had symptomatic relief and remission, while the 27 year old was yet to commence chemotherapy.

Discussion: Most cases of CD go undiagnosed or mostly misdiagnosed for other common causes of lymphadenopathy in Zambia. These are the first case reports in our hospital, with notable backgrounds of physicians having suggested TB treatment from the referring facilities.

Conclusion: Both HHV-8 and none-HHV-8 associated CD may be very common in our population, and there is need for prompt biopsy, reporting and treatment of all patients presenting with lymphadenopathy in our hospitals.