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Context: Castleman disease (CD) is a heterogeneous nonmalignant lymphoproliferative disorder characterized by lymphadenopathy with unique histological features and associated with cytokine-driven constitutional symptoms and biochemical disturbances. Although unicentric (UCD) is curable with complete surgical excision, its multicentric (MCD) counterpart is a considerable therapeutic challenge. Objective: The case study addressed in this report is to determine the application of the recent international consensus diagnostic criteria and treatment guidelines for the management of this complex orphan disease. Design: This was an observational study from August 2018 till now. Setting: It was carried out in the Hematology unit at Oncology Center Mansoura University (OCMU). Patients: A 19-year-old, HIV negative/HHV-8-unknown idiopathic MCD-not otherwise specified (iMCD NOS) vs. unresectable-UCD, female patient with active disease course, presented with fatigue, night fever, hepatosplenomegaly, MHA and severe neutropenia. Interventions: CT scans showed massive mediastinal lymphadenopathy. Biopsies revealed multiple hyperplastic lymphoid follicles with prominent germinal centers and dense plasma cell infiltrates, consistent with CD-plasma cell (PC) variant. Main outcome measures: Exclusion of autoimmune diseases, such as SLE and RA, that mimic iMCD was done. An overlapping IgG4-related disease (IgG4-RD) was also excluded after further IHC study. Our patient did not fulfill the criteria of iMCD-TAFRO syndrome. The platelet count was often increased or normal, there was no myelofibrosis or megakaryocytic hyperplasia, neither anasarca nor renal failure. Other malignant/LPD as lymphoma or MM were excluded. Results: The patient had a partial response to rituximab (375mg/m 2) 8 weekly infusions with corticosteroids as regard regression of lymphadenopathy; confirmed by low metabolic activity on PET-CT (SUV=1.7), and she experienced complete remission of her symptoms and normalization of her biochemical panel. The patient got pregnant after finishing her therapy, a challenging situation, so she is on close monitoring for any worsening or relapse. Conclusions: Diagnosis and identification of CD subtypes are difficult. Improved understanding of the biology affects the decision of disease management. In general, it is recommended to avoid cytotoxic chemotherapy as long as the patient does not have severe disease with progressive organ dysfunction. Rituximab should be considered as an alternative to anti–IL-6 mAb. |
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