Hemophagocytic Lymphohistiocytosis secondary to Rickettsial infection: A case report

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a rare life‐threatening condition characterized by widespread activation of the immune system leading to tissue damage all over the body. It is divided into primary HLH due to inborn error in lymphocytes, T cells, and macrophages and secondary HLH which is mostly due to infections, systemic connective tissue diseases, and lymphoid malignancies. Here, we report a 34‐year‐old man with a history of high‐grade fever, chills, and rigor, eschar, splenomegaly with the laboratory findings of thrombocytopenia, hypochromic RBCs with anisocytosis and basophilic stippling, elevated transaminases, and a positive Weil Felix test along with positive PCR results for Orientia tsutsugamushi and the presence of IgG and IgM antibodies. A detailed workup was done to rule out other etiology for fever. Diagnosis of HLH secondary to Rickettsia infection was made with a thorough history, clinical evaluation, and a variety of investigations. The patient was treated with Doxycycline, Ciprofloxacin, Etoposide, and Dexamethasone but unfortunately, the patient died during treatment due to multiorgan failure. Patients with scrub typhus typically respond well to therapy; therefore, early detection and antibiotic treatment can help avoid serious complications. Scrub typhus with the hemophagocytic syndrome can result in DIC and multiorgan failure. Despite its rarity, scrub typhus may be lethal; as a result, practitioners must be aware of the necessity of detecting and treating suspected cases as soon as possible. We learned that a systematic diagnostic approach, use of diagnostic criteria, and prompt treatment are very crucial in this disease.

Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases

“…The following were excluded from the analysis: articles published in languages other than English; case reports not providing an appropriate amount of information, or where the diagnosis was not certain; and those reporting non-human subjects. The final number of articles included was 69, which resulted in a total number of 98 patients ( Figure 1 ) [ 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , ...…”

“…Characteristics of patients who died in our systematic review (references:[18,22,[24][25][26]42,43,48,49,59,62,71,82]). …”