Clinical benefit of R-CHOP without splenectomy in stage I primary splenic diffuse large B-cell lymphoma

Primary splenic diffuse large B-cell lymphoma (PS-DLBCL) is a relatively rare malignancy, and there are no optimal approaches for diagnosis and management. There are less invasive splenic biopsies that effectively obviate diagnostic and elective splenectomies. We report a man in his 50s with 2-day history of pain in the abdomen and who was found to have a splenic mass on PET-CT. A CT-guided core needle splenic biopsy confirmed the diagnosis of PS-DLBCL. He was managed with six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) alone, without splenectomy. The patient attained complete remission, and he is disease free at 6 years of follow-up.

Rare but Potentially Fatal Presentations of Diffuse Large B-cell Lymphoma: Leukemic Phase or Hemophagocytic Syndrome in Bone Marrow

Diffuse large B-cell lymphoma (DLBCL) is a type of non-Hodgkin Lymphoma commonly presenting as a solid tumor either by nodal or extra-nodal manifestations. Here we describe two atypical presentations of lymphoma, finally resulting in the diagnosis of DLBCL. Case 1: A 53-year-old man with a previous history of nasopharyngeal carcinoma presented with a two-week history of B-symptoms and hyperleukocytosis. Peripheral blood film showed 78% abnormal mononuclear cells. Immunohistochemical stain showing Ki-67 of 90%, negative c-myc, BCL2 and BCL6, and negative c-MYC with fluorescence in-situ hybridization studies on the trephine biopsy, concluded the diagnosis of CD5+ DLBCL of ABC subtype. He received intravenous cyclophosphamide and oral prednisolone for cytoreduction, followed by 6 cycles of chemo-immunotherapy. However, he succumbed due to severe sepsis after the completion of therapy. Case 2: A 56-year-old lady who was initially investigated for pyrexia of unknown origin was noted to have hemophagocytosis upon bone marrow aspirate examination. The bone marrow trephine biopsy revealed some atypical clusters of B-cells positive for CD20 which was inconclusive. PET-CT scan noted an enlarged hypermetabolic spleen without lymphadenopathy. Splenic biopsy with immunohistochemical studies revealed DLBCL of ABC subtype. The diagnosis was consistent with primary splenic DLBCL. She became unwell post splenic biopsy and was admitted to the intensive care unit where she passed away 2 weeks later from Candida and Sternotrophomonas septicemia. These cases highlight the atypical presentations of a common subtype of NHL in our center. Arriving at the definitive diagnosis can be difficult especially when patients are acutely ill, hampering the necessary invasive procedures for diagnosis. The outcomes of both cases are briefly discussed hoping to spread awareness among clinicians on the rare and acutely critical presentations of DLBCL.

Granulocyte Colony Stimulating Factor (G-CSF) Induced Splenic Infarction in Breast Cancer Patient Treated with Dose-Dense Chemotherapy Regimen

Introduction. Granulocyte colony-stimulating factor (G-CSF) is commonly used for prevention and treatment of febrile neutropenia among solid tumor patients. It is considered an effective and relatively safe supportive care medication; however, it can cause rare and serious side effects such as spleen rupture or infarction. Case Presentation. We are reporting a case of a 27-year-old female with breast cancer who has been treated with dose-dense chemotherapy and received colony-stimulating factor as primary prevention of febrile neutropenia that was complicated halfway through with splenic infarction. This finding was confirmed by computed tomography (CT) scan and splenic biopsy. Management was conservative without the need of surgical intervention. Conclusion. Although splenic infarction is an extremely rare side effect of G-CSF, it can be a serious complication that should be recognized, monitored, and managed carefully.

Reticuloendothelial System: Vascular, Nonvascular, and Oncologic

The reticuloendothelial system is the portion of the immune system consisting of phagocytic cells found in reticular connective tissue in the spleen, liver, lungs, bone marrow, and lymph nodes. For the purposes of this chapter, the discussion will be limited to interventions within the spleen and the lymphatic system. Splenic arterial interventions are performed to treat a variety of clinical conditions, including abdominal trauma, hypersplenism, splenic arterial aneurysm/pseudoaneurysm, portal hypertension, and splenic neoplasm, and they provide an alternative to open surgery. Although not commonly performed, percutaneous splenic biopsy and drainage are relatively safe and efficacious procedures. Lymphangiography is a therapeutic option for patients with chylothorax, chylous ascites, and lymphatic fistula. Percutaneous thoracic duct embolization (TDE) is an alternative to surgical ligation of the thoracic duct (TD).

Percutaneous Image-Guided Biopsy of the Spleen

Percutaneous spleen biopsy has a small but important role in the diagnostic approach to splenic lesions. Nonetheless, there remain concerns about the safety of the procedure, limiting its use despite evidence that splenic biopsy performed with optimum technique has comparable diagnostic yield and safety as other solid organ biopsies. To assure appropriate use of percutaneous image-guided spleen biopsy, we discuss the rationale, technique, and outcomes of the procedure.

Autoantibodies in a Three-Year-Old Girl with Visceral Leishmaniasis: A Potential Diagnostic Pitfall

Visceral leishmaniasis (VL), a life-threatening parasitic infection, is endemic in the Mediterranean region. Diagnosis of VL is based on epidemiologic, clinical, and laboratory findings. However, sometimes, clinical features and laboratory findings overlap with those of autoimmune diseases. In some cases, autoantibodies are detected in patients with VL and this could be a potential diagnostic pitfall. In this study, we have reported on a three-year-old girl from a VL-endemic area in Iran, who presented with prolonged fever and splenomegaly. Bone marrow examination, serologic tests, and the molecular PCR assay were performed; however, results were inconclusive. The levels of anti-double stranded DNA, cytoplasmic antineutrophil cytoplasmic autoantibody, and perinuclear antineutrophil cytoplasmic autoantibody were elevated and, at the end, splenic biopsy was performed. The splenic tissue PCR test detected the DNA ofLeishmania infantum. The patient’s condition improved with anti-Leishmaniatherapy, and the autoantibodies disappeared within the following four months. Clinical presentations and laboratory findings of VL and autoimmune diseases may overlap in some patients.