An 8-Year-Old Boy With Fever, Splenomegaly, and Pancytopenia

Abstract

An 8-year-old boy with no significant past medical history presented to his pediatrician with 5 days of fever, diffuse abdominal pain, and pallor. The pediatrician referred the patient to the emergency department (ED), out of concern for possible malignancy. Initial vital signs indicated fever, tachypnea, and tachycardia. Physical examination was significant for marked abdominal distension, hepatosplenomegaly, and abdominal tenderness in the right upper and lower quadrants. Initial laboratory studies were notable for pancytopenia as well as an elevated erythrocyte sedimentation rate and C-reactive protein.Computed tomography (CT) of the abdomen and pelvis showed massive splenomegaly. The only significant history of travel was immigration from Albania 10 months before admission. The patient was admitted to a tertiary care children's hospital and was evaluated by hematology-oncology, infectious disease, genetics, and rheumatology subspecialty teams. Our multidisciplinary panel of experts will discuss the evaluation of pancytopenia with apparent multiorgan involvement and the diagnosis and appropriate management of a rare disease.

Hemophagocytic Lymphohistiocytosis Associated With Hemolytic Uremic Syndrome in a Child: A Case Report and Systematic Literature Review

“…Less frequently, Langerhans cell histiocytosis, neuroblastoma, Wilms tumor, and hepatoblastoma can also manifest with these findings. 24,25 Systemic infectious diseases cause organomegaly by inciting an inflammatory response. The differential diagnoses include a number of viral infections, such as Epstein-Barr virus, CMV, viral hepatitis (hepatitis A, B, C, etc.…”

“…24,26 Congestive causes of hepatosplenomegaly are splenic vein thrombosis, and liver diseases such as portal hypertension and congestive heart failure. 25 We performed BMA on this patient since there are cases of a defined HLH triggered by viral infections; furthermore, hematologic malignancies are common in this age group. 28,29 BMA showed evidence of hemophagocytosis.…”

“…The most common oncologic diseases that present with fever, pancytopenia, and hepatosplenomegaly are leukemias and lymphomas. Less frequently, Langerhans cell histiocytosis, neuroblastoma, Wilms tumor, and hepatoblastoma can also manifest with these findings 24,25. Systemic infectious diseases cause organomegaly by inciting an inflammatory response.…”

Non-Endemic Leishmaniases Reported Globally in Humans between 2000 and 2021—A Comprehensive Review

“…Among travelers, most people were tourists, traveling for work or visiting friends and relatives; only three VL cases were described as military personnel: two deployed in Afghanistan [21] and one in Bangladesh [19]. Refugees were from Somalia (n = 17; [16]) and Ethiopia (n = 1; [17]), while migrants with VL were from Europe and Central Asia (n = 19; [25,36,44,60,208,214,216]), Sub-Saharan Africa (n = 4; [20,28,29]), Latin America (n = 2; [55,199]) and Middle East (n = 2; [10,20]). In one case, a transplacental transmission was described [45].…”

“…Patients were diagnosed in 22 countries, mostly in Europe (n = 434). Outside the European region, cases were diagnosed in Australia (n = 10, [28,36,45,57,64,211,251]), Kenya (n = 16, [16]), Kuwait (n = 36, [19]), Saudi Arabia (n = 35, [259]) and the USA (n = 8, [21,43,49,51,55,60,215]). The predominance of cases from the Mediterranean region likely reflects more intensive travel to southern Europe, especially in people from countries where most cases were diagnosed such as France (n = 107, [6,18,61,68]), the UK (n = 80, [22,38,50,52,58,201,204,207,210,214,219,252,253,261]) and Germany (n = 68, [8,20,25,27,33,35,37,40,44,46,47,53,…”