Clinicopathologic features of castleman’s disease : experience at King Hussein medical center

Abstract EN

Objective: To study the clinicopathologic features of Castleman’s disease (CD), to review the treatment challenges in a group of Jordanian patients at King Hussein Medical Center, and to compare that with international data.

Methods: This is a retrospective review of CD cases conducted at King Hussein Medical Center over 8 years (January 2009 to December 2016).

A total of 21 cases of histopathologically diagnosed CD were enrolled in this study.

Clinical data and histopathological parameters were analyzed and correlated among different subtypes of the disease with different outcomes and associations.

Results: There were 14 males and 7 females.

The median age of presentation was 40.1 years.

Common symptoms include lymphadenopathy 76%, anemia 35%, abdominal pain 30%, splenomegaly in 19% of cases.

Lymphoma was diagnosed in 10% of cases.

The disease was localized in 15 cases and multicentric in 6 cases.

Univariate analysis showed that most of multicentric CD cases presented with complications compared to localized disease (p value of 0.0002).

Of multicentric CD, 2 cases were positive for Human Herpes Virus-8 (HHV-8).

The results of Human Immunodeficiency Virus (HIV) were available in 15 patients, and no case was positive.

Hyaline vascular morphology was the commonest histopathological pattern observed in 67%, followed by plasma cell 19%, and mixed type in 5%.

For all patients with localized disease, hyaline vascular was the only pathologic variant, whereas 4 out of 6 (66.6%) multicentric CD were of plasma cell type.

Treatment of unicentric CD consisted of surgical resection, whereas for multicentric it was medical and 19 patients were followed up.

Of these, 93.3% of unicentric disease remain symptom free without recurrence, while 2 cases of multicentric CD died, and the 3 others attained partial remission .

Conclusion: Unicentric and multicentric CD are different clinical entities with overlapping histologic features.

Most of the cases of Jordanian patients with CD exhibit an indolent clinical course with local surgical therapy.

Further studies are needed to further elucidate the epidemiology, pathogenesis, clinical behavior, and optimal therapeutic regimens of this rare disease.