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.