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Hypereosinophilia as a presenting feature of anaplastic large cell lymphoma

A 67-year-old man, with a past history of homozygous MTHFR mutation complicated by venous thrombosis, was referred to a hospital for severe eosinophilia [white blood cell count (WBC) 21Æ5 · 10/l with neutrophils 9Æ46 · 10/l, eosinophils 9Æ67 · 10/l and normal erythrocyte and platelet counts]. Investigations (particularly for parasites, allergies, pulmonary disease, FIP1L1PDGFRA fusion and T-cell receptor gene rearrangement) were negative, and the diagnosis of idiopathic hypereosinophilic syndrome was made. He was then treated with steroids for 5 months; the treatment was partially effective. He was readmitted 5 months later for fatigue associated with decreased consciousness. The WBC was 25Æ5 · 10/l with 15Æ5 · 10/l eosinophils, 8Æ4 · 10/l neutrophils and normal erythrocyte and platelet counts. The spinal fluid was cloudy and aseptic, with 3 · 10/l leucocytes including 90% eosinophils and 10% pleomorphic, multinucleated cells with large prominent nucleoli (figures). These dysmorphic cells expressed CD30 and CD25 on immunophenotyping. Karyotyping failed because of lack of cell growth. At the same time, a chest X-ray showed endobronchial thickening, which histopathological studies showed to be a CD30-positive anaplastic lymphoma kinase-negative lymphoproliferative disorder. The diagnosis of anaplastic large cell lymphoma with meningeal localization was made. Unfortunately, the patient died shortly after the first session of chemotherapy. This case emphasizes that it is sometimes necessary to perform repeated exhaustive investigations, especially for lymphoproliferative disorders, in the case of unexplained chronic eosinophilia.