2 | Case report
A 34 years old male presented to the emergency department with a history of high-grade intermittent fever, associated with chills and rigor along with a frontal headache with no rash or bleeding diathesis. There is no history of cough, sore throat, pain abdomen, burning micturition, earache/discharge, nausea, vomiting. There was the presence of two similar dark scab-like lesions with erythematous bases around 1 cm in diameter on the abdomen (Figure 1). He was later admitted for further evaluation and a definitive diagnosis. In view of thrombocytopenia (platelet count – 97600/cmm, raised transaminases (AST – 164 IU/dl and ALT – 156 IU/L), LDH (i.e2780 IU/L), CRP (i.e1474 mg/L) with no other localizing signs, and a positive Weil Felix Test he was treated as a case of Rickettsia fever with Doxycycline and Ciprofloxacin tablets. He was hemodynamically stable but continued to have fever(101-102°F) with chills and rigors which was intermittent. The patient was receiving supportive care, including IV fluids and paracetamol for fever. The fever began to fade during his stay, but it was predicted to last for around a month, according to the infectious diseases specialist.
He was evaluated in the line of infective, inflammatory, autoimmune, neoplastic possibilities for the fever. To rule out infectious processes as a cause for fever; PPD tests and malarial blood smear were done for Tuberculosis and Malaria which were negative. Similarly, workup was done to rule out EBV, CMV, Hepatitis A, Hepatitis B, Hepatitis C, HIV 1 and 2, Dengue fever, Salmonella, and Brucella infections which were negative.
Then, a Peripheral blood smear was done where Hypochromic RBC with Anisocytosis and basophilic stippling along with-it Leukopenia and giant platelets were seen. For further evaluation, a Bone marrow biopsy was done which showed Dys-erythropoietic features with erythrophagocytic cells (Figure 2). In the line of investigation to rule out malignancy; a CT scan of the Chest, Abdomen, and pelvis was done. An Abdomen CT scan showed a spleen with an upper limit size measuring 14 cm. Whole Body FDG PET/CT was done which showed mildly enlarged spleen measuring up to 13 cm in length, demonstrating mild diffuse hypermetabolism of liver activity. The remaining low-attenuation splenic lesions were not appreciated with certainty on the current unenhanced CT, without focal abnormal FDG uptake. However, few small upper abdominal lymph nodes are seen demonstrating faint FDG uptake just above background liver activity, including at the gastrohepatic ligament, periportal, and right superior diaphragmatic regions, such as a 12 x 8 mm gastrohepatic ligament lymph node. There is normal FDG activity throughout the remainder of the abdomen and pelvis.
After this extensive Work-up, a diagnosis of Hemophagocytic lymphohistiocytosis secondary to Rickettsial infection was made with the following findings:Triglyceride: 267 mg/dl, Ferritin: >2000 mcg/dl, Fibrinogen: 98mg/dl, Bone marrow aspiration and biopsy: Hyperactive macrophages with erythrophagocytosis, LDH: 2780 U/L, Bi-cytopenia: Hb:10.9g/dl TLC: 3800/microliter
He was started on dexamethasone 10 mg/m2 after which the patient started getting better. Further treatment were dexamethasone 10 mg/m2 once daily for 2 weeks followed by tapering to 4-5 mg/ m2, Etoposide 150 mg/m2twice weekly for 6 weeks followed by once weekly and reassessment accordingly. The patient then developed abdominal pain in the right and left upper quadrant along with petechial spots in the abdomen. He also developed a high-grade fever of 103 F along with features suggestive of septic shock. He was shifted to ICU due to his deteriorating condition and deranged hematological panel. During his stay in ICU the patient died because of MODS.