Abstract
Autoimmune cytopenia, a known paraneoplastic complication of lymphoid neoplasms, may occur before, concurrently, at relapse, or even years after completion of lymphoma treatment. In the case of Hodgkin lymphoma (HL), it is thought that immune dysregulation, typical of this neoplasm, may be involved in the genesis of these manifestations. We report a 57-year-old male presenting with stage IIIA, International Prognostic Score (IPS) 4, nodular sclerosis HL, and severe AA (SAA) confirmed on the histologic exam of the bone marrow that showed severe marrow hypoplasia due to a decrease in the elements of the three cell linages with left shift of the myeloid maturation. Immunosuppression with steroids and cyclosporine A was started. Eltrombopag and G-CSF were also added. In spite of prompt initiation of immunosuppressive therapy, the patient presented an unfavorable outcome with progressive pancytopenia and severe acute cerebral hemorrhagic event. The patient died 59 days after admission. Although autoimmune disorders are described in HL, its concomitant diagnosis is extremely rare. Our case shows a rare instance of SAA as the first manifestation of HL.
Highlights
Hodgkin lymphoma (HL) accounts for approximately 10% of all malignant lymphomas, commonly affecting young adults. It usually presents with localized disease involving cervical, supraclavicular, and mediastinal regions. e classical form represents about 95% of the HL, nodular sclerosis being the most frequent subtype [1]
Aplastic anemia (AA) is a hematopoietic stem cell disorder defined by reduced bone marrow cellularity and impaired peripheral blood cells production [3, 4]
Classical HL is characterized by the presence of B cells derived from germinal center, called Hodgkin or Reed–Sternberg cells (HRS)
Summary
Hodgkin lymphoma (HL) accounts for approximately 10% of all malignant lymphomas, commonly affecting young adults. Severe aplastic anemia (SAA) comprises bone marrow cellularity less than 25% and depression of at least two of the three hematopoietic lineages (absolute neutrophil count (ANC)
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