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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

7 responses to “Hacket 4 splenomegaly with fever in a small child having normal Hb electrophoresis”

  1. Nfor Leonard avatar

    Hello

    Interesting case

    Though far from my usual daily clinical cases but I think a blood neoplasia should be among your differentials too. Acute myeloid leukemia? Other?
    FBC has 2 elements already anemia and thrombocytopenia and you have lymphadenopathy and splenomegaly

    1. admin avatar

      Thanks for the insight and reflections Doctor. We are still waiting for peripheral blood smear to check myeloblasts and other shapes of cells but we are surprised by the regenerative anemia which seems to point to a reactive bone marrow unlike the usual aregenerative in leukemia. Yet we are all waiting for the results of the peripheral blood smear.

    2. Germaine Mbange avatar

      Thanks Doctor . I was thinking in that same light when I saw a pancytopenia

  2. TetuAl avatar

    Waooh, interesting case. I could put my hand in fire that the terrain is sickle cell anemia….just to read a EHb AA. If we push our thinking further, can we rely on this test 100%?

    I won’t have suspected a type 1 DT….given that the guy isn’t an avid eater from history, and we have no notion of polydispia nor polyuria.

    Did a blood smear test or osmotic fragility test exclude a possible hereditary spherocytosis HS? Though being an autosomal dominant disease, one will expect an parent to have similar ailment. We shouldn’t forget to that the gene for HS has incomplete penetrance too!

    I am curious to know more about “hyper-reactive malaria syndrome”.

    Doc what about a stool exam, a simple test that could guide too.

    I am anxious to know how the case evolves, may he get well soon..

    1. admin avatar

      Haha..Hand in fire right? I was hoping for at least a variant if not the typical SS. Results of peripheral blood smear still awaited. Well Type 1 DM feels far-fetched especially with the splenomegaly… We were hoping for blood in urine to indicate schistosomiasis too. Hopefully one could also consider stool or a specific antibody test. Check hyper reactive malarial syndrome (or tropical splenomegaly symdrome), suggested as an exaggerated immune response to repeated or chronic exposure to malaria. See more on it here: https://emedicine.medscape.com/article/959720-overview-overview?src=mbl_msp_iphone&ref=whatsapp

  3. AVANI Stephanie avatar

    For me from the history, clinical and paraclinical findings.I think more of tropical splenomegaly due to repeated poorly treated malaria

    1. admin avatar

      Thanks for the reaction, Dr. We hope to be able to prove it.

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