Hacket 4 splenomegaly with fever in a small child having normal Hb electrophoresis

A 5-year old boy was brought by mum for fever and abdominal pains since 4 days. The boy first had a fever which became associated to peri-ombilical abdominal pains the following day. There are no known factors that aggravate or relieve the pains, and further characteristics of the pain could not be explained. Child was administered albendazole 400mg and Paracetamol Dose for weight the night before consultation.
His mother mentioned similar episodes of abdominal pains over the years usually preceded by fever, no jaundice noticed, usually treated in health centres. A previous abdominal ultrasound done the previous year showed splenomegaly of about 11.8cm.
His Hb electrophoresis is unknown likewise that of his parents. Never had a glycemia taken. HIV serology negative done the previous year. He is the 1st out of 3 children and the only boy. No known chronic disease or frequent illnesses in any of siblings. Psychomotor development seems adequate for age.
On clinical exam, there is fatigue, conserved appetite (never been very interested in food), low weight gain, dysuria, concentrated yellow urine, no enuresis nor nocturia, stool aspect could not described. He has moderate palour of conjunctiva, palms and feet. White sclera. Bilateral cervical palpable lymph nodes, about 0.5cm in diameter. Tenderness on deep palpation of ombilical region. Hacket 3 splenomegaly firm with smooth borders, and hepatomegaly about 2.5cm below right costal margin. A dry scarring ulcer on the big toe of right foot. The rest of physical examination was unremarkable.

STOP. THINK. DIAGNOSIS…

We therefore retain a fever, abdominal pain and a reactive or hyper reactive spleen.

Working Diagnosis was Urinary tract reaction on a terrain to be investigated. Differential: Malaria or sepsis of another portal of entry, on a terrain to be investigated.
For the terrain or underlying condition: Hemoglobinopathy with first option being Sickle Cell Disease, then hereditary spherocytosis.
As differential: G6PD deficiency, malignant hemopathy, Portal Hypertension, or auto immune disease, ketoacidosis in Type1 Diabetes Mellitus, and an associated gastritis.
Work-ups were requested in two shifts. Available work ups:
FBC with pancytopenia (anemia is normocytic, there is lymphopenia and moderate thrombopenia). Reticulocyte count showing regenerative anemia, Normal levels of G6PD, Positive RDT for malaria, with trophozoites of Pfalciparum on thick blood smear, urine dipstick with no abnormal findings, Hb electrophoresis AA.

The patient was treated for simple malaria and Mum was told to complete deworming with second dose of Albendazole 400mg 1 week later. There was regression of fever and abdominal pains when reviewed few days later.
The terrain is still being discussed.
Peripheral blood smear, liver enzymes(SGPT, SGOT), H.pylori serology and an abdominal ultrasound are awaited.

Please do discuss and send in your suggestions for diagnosis and reflections as this would greatly help with the next step. The patient is being followed up at an out-patient unit though in a context of traditional ‘rites’ being carried out on him by family.

We are also, now considering, specifically the possibility of hyper reactive Malarial syndrome or other parasitic invasion of spleen  if the other tests are negative.

Thanks for your participation and contributions below.?

Presented by Dr.P.O