Jebmh.com Case Report J. Evid. Based Med. Healthc., pISSN- 2349-2562, eISSN- 2349-2570/ Vol. 3/Issue 80/Oct. 06, 2016 Page 4374 SLE ASSOCIATED HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS WITH DISSEMINATED HISTOPLASMOSIS IN A HIV SEROPOSITIVE PATIENT Pranati Mohanty 1 , Pallavi Bhuyan 2 , Asaranti Kar 3 , Pradeep Kumar Behera 4 , Chandra Sekhar Mohapatra 5 1 Assistant Professor, Department of Pathology, S.C.B. Medical College, Cuttack, Odisha. 2 Assistant Professor, Department of Pathology, S.C.B. Medical College, Cuttack, Odisha. 3 Associate Professor, Department of Pathology, S.C.B. Medical College, Cuttack, Odisha. 4 Assistant Professor, Department of Pathology, S.C.B. Medical College, Cuttack, Odisha. 5 Assistant Professor, Department of Pathology, S.C.B. Medical College, Cuttack, Odisha. ABSTRACT BACKGROUND Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon, life-threatening and likely underdiagnosed disease of diverse aetiologies caused by a defective NK/T - cell cytotoxic pathway resulting in uncontrolled hypercytokinaemia leading to end organ damage carrying a high mortality rate. Here, we report HLH complicating SLE in a 42 years old female associated with disseminated histoplasmosis and accidentally detected as seropositive for HIV. This is extremely rare in the world literature. KEYWORDS Hemophagocytic lymphohistiocytosis, histoplasmosis, SLE. HOW TO CITE THIS ARTICLE: Mohanty P, Bhuyan P, Kar A, et al. SLE associated hemophagocytic lymphohistiocytosis with disseminated histoplasmosis in a HIV seropositive patient. J. Evid. Based Med. Healthc. 2016; 3(80), 4374-4376. DOI: 10.18410/jebmh/2016/930 INTRODUCTION: Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon life-threatening hyperinflammatory syndrome caused by hypercytokinaemia due to a highly stimulated, but ineffective immune process. The underlying pathogenic mechanism being a defect in the NK/T-cell cytotoxic pathway leading to over activation of macrophages resulting in hemophagocytosis in various organs. At the same time, this dysregulated immune system triggered by several factors results in a cytokine storm leading to multiorgan dysfunction. [1] HLH has been traditionally divided into a primary form, which typically manifests in children with documented genetic abnormalities of the cytotoxic function of NK/T cell and a secondary form that tends to occur at older ages in the setting of an associated condition such as infection and malignancy without an identifiable genetic abnormality. [2] Disseminated histoplasmosis, an opportunistic fungal infection has been reported to be associated with Hemophagocytic Syndrome (HPS) in HIV positive and AIDS patients. [3] But, its association with SLE has been rarely reported. [4] The present case who was under treatment for SLE suddenly developed MAS with disseminated histoplasmosis found to be seropositive for HIV, which is reported here because of a rare and unique association. CASE REPORT: A 42 years old female presented with skin rash, photosensitivity and alopecia one and a half years back. Lupus panel showed positivity for ANA, dsDNA and SmAg with complement C3-65 mg/dL (Ref: 90-180) and C4- 17.2 mg/dL (Ref: 10-40). She was diagnosed as SLE and started Omnacortil 10 mg daily with clinical improvement. After 6 months, she developed low-grade fever with an evening rise of temperature for 10-12 days. She had mild pallor, liver 3 cm enlarged and spleen not palpable. Haematological investigations revealed pancytopenia (Hb- 8.4 gm/dL, TLC-1,500/cmm, TPC-17,000/cmm), DC: N- 86%, L-10%, M-3%, E-1%, B-0% and ESR 10 mm/1 st hr. She had ulceration in the mouth and developed bleeding per rectum. Viral serology for dengue, hepatitis B and C were all negative. ICT for malaria and Widal test were also negative. Biochemical investigations revealed blood urea 36 mg/dL, S. creatinine 1.0 mg/dL, S. bilirubin (D)-0.3 mg/dL, (T)-0.8 mg/dL, SGOT-128 IU/L, SGPT-82 IU/L, S. Alk. Phosphatase- 146 IU/L (<310 Ref), S. Na+-138 mEq/L, S. K+-4.1 mEq/L. Bone marrow examination revealed extremely hypocellular marrow with marked proliferation of macrophages (Fig. 1) showing prominent hemophagocytosis (Fig. 2), also stuffed with numerous capsulated yeast forms of Histoplasma capsulatum confirmed by PAS stain (Fig. 3). Serum ferritin, triglyceride and LDH were 24,560 mg/dL, 460 mg/dL and 840 mg/dL, respectively. Thus, a diagnosis of hemophagocytic syndrome/HLH with disseminated histoplasmosis was made. Immediately Inj. Amphotericin B 1 gm/kg IV 8 hrly. was started. She was detected strongly positive for HIV with CD4 cell count of 11/cmm and viral load of 1 crore copies/mL. In spite of IV haemostatics and antibiotics PR bleeding did not stop. Colonoscopy showed multiple mucosal ulcerations with pool of blood. Further investigations revealed PT-11.7 secs. (C), >1 min. (T), APTT-27.6 secs. Financial or Other, Competing Interest: None. Submission 12-09-2016, Peer Review 17-09-2016, Acceptance 30-09-2016, Published 06-10-2016. Corresponding Author: Dr. Pranati Mohanty, Assistant Professor, Department of Pathology, S.C.B. Medical College, Cuttack, Odisha. E-mail: drpranati@live.com DOI: 10.18410/jebmh/2016/930