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  • br Case report A year old female presented with splenomegaly

    2019-05-10


    Case report A 50-year-old female presented with splenomegaly (24 cm) and pancytopenia in Mar. 2014. A bone marrow biopsy revealed hypocellular marrow (<5%). Biochemistry tests including ANA and anti-Ds-DNA were normal. Flow cytometry revealed no CD59/CD55 deficient clones. Aplastic anemia was diagnosed after serial work-ups. She initially received low dose prednisolone (10 mg/day). The hemogram improved but worsened again in Dec. 2014. On examinations showed white cell count (WBC) 810/ul; hemoglobin 7.0 g/dl; platelet count 64,000/ul, and lactate dehydrogenase 1041 U/L. An abdominal computed tomography revealed extensive lymphadenopathies mainly at retroperitoneum and massive splenomegaly, with biopsy proved DLBCL (Fig. 1). Repeated bone marrow biopsy again showed remarkable hypocellularity (Fig. 2). Concerning for profound myelosuppression, we delivered chemotherapy with standard dose R-COP regimen (rituximab; cyclophosphamide; vincistine, and oral prednisolone), and we skipped anthracycline for the first 3 cycles due to concern for profound marrow suppression. Long-acting filgrastim (pegylated granulocyte colony stimulating factor, [G-CSF]) was given 24 h after chemotherapy. The patient stood 3 cycles of R-COP very well and there was no occurrence of neutropenia. Interestingly, her WBC recovered shortly after the 1st example of R-COP (Fig. 3). Further 5 cycles of cytotoxic therapy with standard dose R-CEOP (rituximab; cyclophosphamide; epirubicin; vincistine, and oral prednisolone) regimen were given, and a follow-up abdomen CT showed marked decreased spleen size and resolution of previous lymphadenopathy (Fig. 4). PET-CT confirmed complete metabolic response (Fig. 5). We accomplished successful stem cell harvest after documented recovery of hematopoiesis (Fig. 6). Her HLA-DR typing revealed DRB1*12.
    Discussion Immunological manifestations occasionally develop concurrently with lymphoid neoplasms, including immune thrombocytopenia and autoimmune hemolytic anemia, but rarely acquired AA. Since Keisu et al notified one third of patients with initial diagnosis of acquired idiopathic AA developed myelodysplastic syndrome (MDS) or Non-Hodgkin lymphoma (NHL), there have been more and more studies finding chronological associations between AA and NHL: some cases developed NHL during IST for initially diagnosed AA. In literature review, NHL tends to occur shortly after IST for antecedent AA, predominantly 2–4 months after IST. B-cell lymphomas are more likely. Dorr et al found withdrawal of IST plus radiotherapy attain complete remission. Suzuki et al used anthracycline-based chemotherapy to achieve a partial remission; while Saitoh et al reported a case that followed a more fulminant course and showed no response to IST withdrawal or chemotherapy, which might be the nature of a late stage relapsing lymphoma. On the other hand, NHL could also developed after a protracted use of IST, often 1.5 years later, in which T-cell or B-cell neoplasms evolved equally, and responded poorly to chemotherapy, carrying a grim prognosis. HLA-DR15 might account for the paraneoplastic relationship between lymphoproliferative disease (LPD) and preceding AA: Nissen et al reported a case of NHL with tumor regression completely 10 days after diagnosis, accompanied with progressive pancytopenia, without treatment. The patient was positive for HLA-DR15; Jerez et al found a higher frequency of HLA-DR15 positivity (75% vs 44%, P = 0.02) in patient with concomitant AA and T large granular lymphocyte leukemia (T-LGL) than those who had no T-LGL clone. Though it is not HLA-DR15 in our case, whether other immunogenic HLA subtypes could predispose to both AA and LPD remains unclear. However, these evidences explained the paraneoplastic nature of AA as a collateral damage to hematopoietic progenitor cell while launching immune attack toward occult lymphoma cells. As occult lymphoma cells tolerate, escape immune surveillance mainly via IST, and re-expand as clonal evolution, it might be more aggressive than de novo lymphoma cells.