Hepatosplenic Lymphoma Presenting Initially as Hemophagocytic Syndrome in a 21-Year-Old Man with Crohn’s Disease: A Case Report and Brief Literature Review

Abstract

The present report describes a case involving a 21-year-old man diagnosed with Crohn’s disease 10 years previously. A diffuse mild to moderate colitis was found at colonoscopy. Different treatments based on 5-aminosalicylic acid, prednisone, methotrexate and purinethol were attempted but did not induce remission. Five years later, a combination of purinethol 25 mg per day and infliximab 350 mg every six to seven weeks proved to be successful. Four years later, the patient was admitted to hospital with fever (39°C) and bicytopenia, without any gastrointestinal symptoms. A complete blood panel revealed the following: white blood cell count (Wbc) 0.9×109/L (neutrophils 0.2×109/L), a hemoglobin level of 156 g/L and a platelet count of 105×109/L. The final doses of purinethol and infliximab were given one month before admission. A bone marrow biopsy was performed and showed hemophagocytic syndrome (Figure 1A). Epstein-Barr virus (EBV) serology (viral capsid antigen [VCA] immunoglobulin [Ig] M and EBV nuclear antigen [EBNA] IgG) was negative and an abdominal computed tomography scan showed mild hepatosplenomegaly. The patient evolved well clinically and the blood count normalized with filgrastim (Neupogen [Amgen Inc, USA]). Figure 1) A Bone marrow biopsy showing increased cellularity (90%), white blood cell hyperplasia and severe hemophagocytosis. A CD68 study revealed histiocyte population infiltration. B Splenic biopsy showing lymphocyte infiltration with irregular nucleus expressing ... One week later, the patient’s blood count showed pancytopenia (Wbc 2.1×109/L, neutrophils 0.1×109/L, hemoglobin level of 133 g/L and a platelet count of 42×109/L) presenting with abdominal pain. A distal endoscopy showed mild proctitis. Due to the pancytopenia, no treatment was started. EBV serology was positive for EBNA IgG and negative for VCA IgM. No antiviral treatment was started. He was discharged, and outpatient chemotherapy with dexamethasone, etoposide and cyclosporin A (hemophagocytic lymphohistiocytosis – 2004 protocol) was initiated. The patient was readmitted two months later due to Escherichia coli sepsis. Chemotherapy was restarted. Septic shock with multiple organ failure ensued and he was transferred to the intensive care unit (ICU). A few months later, when discharged from the ICU, the patient presented with many secondary infections. Another bone marrow biopsy subsequently revealed hepatosplenic lymphoma. EBV was not found on that biopsy. A palliative splenectomy (25.3 cm) was performed, and the cytology showed the same type of lymphoma with the presence of EBV (Figure 1 B). After many trials of chemotherapy and multiple infections, the patient died from Klebsiella ozaenae infection.