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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 103-108 |
| Number of pages | 6 |
| Journal | Journal of clinical and experimental hematopathology : JCEH |
| Volume | 55 |
| Issue number | 2 |
| DOIs | |
| State | Published - 2015 |
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All Science Journal Classification (ASJC) codes
- Medicine(all)
Cite this
Multicentric Castleman Disease with Monoclonal Incomplete IgH Restriction : A Rare Coexistence. / Goyal, Gaurav; Kendric, Kayla; Silberstein, Peter T.; Caponetti, Gabriel C.; Vivekanandan, Renuga.
In: Journal of clinical and experimental hematopathology : JCEH, Vol. 55, No. 2, 2015, p. 103-108.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Multicentric Castleman Disease with Monoclonal Incomplete IgH Restriction
T2 - A Rare Coexistence
AU - Goyal, Gaurav
AU - Kendric, Kayla
AU - Silberstein, Peter T.
AU - Caponetti, Gabriel C.
AU - Vivekanandan, Renuga
PY - 2015
Y1 - 2015
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84979993362&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84979993362&partnerID=8YFLogxK
U2 - 10.3960/jslrt.55.103
DO - 10.3960/jslrt.55.103
M3 - Article
C2 - 26490523
AN - SCOPUS:84979993362
VL - 55
SP - 103
EP - 108
JO - Journal of clinical and experimental hematopathology : JCEH
JF - Journal of clinical and experimental hematopathology : JCEH
SN - 1346-4280
IS - 2
ER -