^{Multicentric Castleman Disease with Monoclonal Incomplete IgH Restriction: A Rare Coexistence}

Abstract

Castleman disease is a rare lymphoproliferative disorder that may have a unicentric or multicentric clinical presentation. Herein we present the case of a 49-year-old female with a 3-year history of progressively worsening lymphadenopathy associated with fevers, chills and night sweats. Laboratory studies showed anemia and mildly elevated sedimentation rate. A computed tomogram scan of the chest, abdomen and pelvis showed multiple enlarged bilateral axillary, supraclavicular, subpectoral, submental, retroperitoneal, and para-aortic lymph nodes. A right axillary lymph node biopsy was performed and found to display histopathologic features compatible with the plasma cell type of Castleman disease. The patient was found to be human immunodeficiency virus (HIV)-positive, with a viral load of 104,000/mL and a CD4 cell count of 84 cells/mm(3). Molecular studies on the lymph node specimen revealed an incomplete monoclonal DH-JH rearrangement in the IgH gene. The patient was initially treated with antiretroviral therapy with a combination of elvitegravir, cobicistat, emtricitabine and tenofovir that improved her fatigue and malaise. As treatment for Castleman disease, she was administered a combination of rituximab and etoposide, which led to a reduction in lymphadenopathy. To the best of the authors' knowledge, this is the first reported case of multicentric Castleman disease with monoclonal incomplete IgH gene rearrangement in an HIV-positive patient.

The application of a multidisciplinary approach in the diagnosis of Castleman disease and Castleman‐like lymphadenopathies: A 20‐year retrospective analysis of clinical and pathological features

“…As in other reports, 10,31,35–41 in our series the presence of a monoclonal IGH rearrangement was not a typical feature of CD and, when present, a possible explanation was given. In the literature, 13 CD have been found to have monoclonal rearranged IGH 35–41 .…”

“…As in other reports, 10,31,35–41 in our series the presence of a monoclonal IGH rearrangement was not a typical feature of CD and, when present, a possible explanation was given. In the literature, 13 CD have been found to have monoclonal rearranged IGH 35–41 . Of these, one was a plasma cell type UCD in which immunohistochemistry for kappa and lambda was not conclusive and no data on the presence of a serum M protein was reported, 35 four were POEMS‐MCD, 37,41 three were iMCD and two of these had EBV DNA copies in the lymph nodes, 38 four had a concomitant haematological disease (two mantle cell lymphoma, one Hodgkin lymphoma and one multiple myeloma) 39 and one was an HIV+ patient with an incomplete IG rearrangement 36 .…”

“…[31][32][33][34] One of our patients was diagnosed with NMZL, in which the CD-like features could either be induced by the clonal B-cell proliferation or represent the background where the clonal proliferation subsequentially developed. As in other reports, 10,31,[35][36][37][38][39][40][41] in our series the presence of a monoclonal IGH rearrangement was not a typical feature of CD and, when present, a possible explanation was given. In the literature, 13 CD have been found to have monoclonal rearranged IGH.…”

“…In the literature, 13 CD have been found to have monoclonal rearranged IGH. [35][36][37][38][39][40][41] Of these, one was a plasma cell type UCD in which immunohistochemistry for kappa and lambda was not conclusive and no data on the presence of a serum M protein was reported, 35 four were POEMS-MCD, 37,41 three were iMCD and two of these had EBV DNA copies in the lymph nodes, 38 four had a concomitant haematological disease (two mantle cell lymphoma, one Hodgkin lymphoma and one multiple myeloma) 39 and one was an HIV+ patient with an incomplete IG rearrangement. 36 In our series, we found a monoclonal IGH rearrangement in 1/12 UCD (8.3%) and in 4/15 MCD (26.7%).…”

A Review of Genetic Abnormalities in Unicentric and Multicentric Castleman Disease

“…B-cell and T-cell clonality were studied in both MCD and UCD cases. These studies reported 11 of 28 cases of MCD (39%) and 1 of 21 cases of UCD (4.8%) with findings of immunoglobulin heavy chain (IgH) gene arrangement (Supplemental Table S3) [8,[29][30][31][32][33][34]. T-cell clonality was reported in three of four cases of MCD (75%) compared to zero of four studied UCD cases (0%).…”