ABSTRACT
Haemophagocytic lymphohistiocytosis (HLH) is a rare condition of uncontrolled immune activation as a result of an inherited genetic defect or in response to malignancy, autoimmune disease, rheumatological disease, AIDS infection or post-transplant immunosuppression. Described here is the case of a 19-year-old Caucasian man who presented with complaints of worsening fever, new-onset jaundice and lethargy after failing treatment for suspected infectious mononucleosis. Physical examination was significant for fever and splenomegaly while laboratory results revealed transaminitis, cytopaenia, indirect hyperbilirubinaemia and elevated ferritin, raising the likelihood of both autoimmune haemolytic anaemia and HLH. He tested positive for Epstein-Barr virus (EBV), and bone marrow biopsy revealed hypercellular marrow with haemophagocytosis and no evidence of malignancy. High dose steroids were initiated with significant improvement in haemoglobin, resulting in a final diagnosis of HLH secondary to acute EBV infection. The patient was discharged on continued high-dose prednisone with planned taper and consideration of outpatient rituximab therapy for 4 weeks. High clinical suspicion and prompt evaluation were critical to early treatment and decreased morbidity.
Subject(s)
Epstein-Barr Virus Infections , Lymphohistiocytosis, Hemophagocytic , Adult , Bone Marrow , Epstein-Barr Virus Infections/complications , Epstein-Barr Virus Infections/diagnosis , Epstein-Barr Virus Infections/drug therapy , Fever , Herpesvirus 4, Human , Humans , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/drug therapy , Lymphohistiocytosis, Hemophagocytic/etiology , Male , Young AdultABSTRACT
A 28-year-old primigravida was evaluated for complaints of difficulty urinating and pelvic pain of 6-weeks duration. She denied fever, night sweats, weight loss or fatigue. Pelvic ultrasonography revealed a single fetal pole with cardiac activity and a 7 cm mass in the anterior vagina which encased the urethra. The diagnosis of diffuse large B-cell lymphoma germinal centre type was made on analysis of biopsied pelvic mass. Whole body MRI revealed the disease was limited to the vagina. The patient received six cycles of Rituximab-cyclophosphamide, doxorubicin, vincristine and prednisone with significant improvement in her symptoms. Serial ultrasounds over the subsequent months showed appropriate development of the fetus. Whole body MRI after treatment showed decreased size and decreased signal of the primary pelvic mass compatible with favourable treatment response. Challenges in the management of this rare presentation of lymphoma are discussed.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Lymphoma, Large B-Cell, Diffuse/drug therapy , Pregnancy Complications, Neoplastic/drug therapy , Vaginal Neoplasms/drug therapy , Adult , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Dysuria , Female , Humans , Lymphoma, Large B-Cell, Diffuse/diagnostic imaging , Magnetic Resonance Imaging , Pelvic Pain , Prednisone/therapeutic use , Pregnancy , Pregnancy Complications, Neoplastic/diagnostic imaging , Rituximab/therapeutic use , Ultrasonography , Vaginal Neoplasms/diagnostic imaging , Vincristine/therapeutic useABSTRACT
We report a case of a small-cell variant of anaplastic large-cell lymphoma, with an unusual clinical presentation mimicking sepsis and a fulminant clinic course, in a 48-year-old Caucasian female. In this report, we discuss the diagnostic challenge, histopathologic features, and unique cytogenetic features of this case, in order to raise awareness of this rare presentation and emphasize the importance of meticulous peripheral smear examination and early bone marrow evaluation.
