A 2 year old child with fever, hepatosplenomegaly and generalised lymphadenopathy

Author: Dr.Pawan

Question
Case History:
A 2-year-old male child born of non-consanguineous marriage hailing from Uttar Pradesh (area endemic for Kala Azar) presented with fever since 1 year (Fever would be almost 101o-102oF every day) and progressive distension of abdomen since 6 months.
Birth History, Milestones, Dietary History and Immunization history were normal.

Examination:
HR = 134/min, all peripheral pulses well felt
RR = 52/min, minimal respiratory distress
· Significant axillary, inguinal and cervical lymphadenopathy.
· Pallor +
· Petechial rash over the trunk +
· Seborrheic dermatitis on the scalp+
· No hyperpigmentation of skin
· Generalized edema +
Anthropometry - s/o chronic malnutrition (Height = 75 cm, Weight = 9 kg.)
Systemic examination: Splenohepatomegaly (firm) with ascitis. Other systems were normal.

Clinical Impression – 1) Kala Azar
2) HIV
3) Disseminated TB
4) Malignancy
5) Histiocytosis

Investigations:
· Hb = 5.7 gm % [MCV = 90.2, MCHC = 28.9, RDW = 17.8]
· WBC = 2,100/cumm (ANC = 252 cell/cumm, ALC = 1848 cells/cumm)
· Platelet count = 49,000/cumm
· Reticulocyte count = 7%
· ESR = 100 mm at end of 1 hour
· Total Proteins = 5.3 gm/dl
Albumin = 2.6 gm/dl
Globulins = 2.7 gm/dl
· Alkaline Phosphatase = 441 IU/L [N = 30-120 IU/L]
· PT/PTT/Bilirubin/Transaminases/electrolytes/creatime/RBS/Cholesterol/Triglycerides/
Calcium = Normal
· Coomb’s test – negative
· PS for material parasite – negative
· HIV ELISA – negative
· Mantoux test – negative
· X Ray Skull = No punched out lesions
· X Ray Chest = Normal
· Brucella IgM & IgG – negative

· USG Abdomen –
Splenohepatomegaly with gross ascitis with bright echotexture D/D- ?Storage disorder.

· Bone Marrow aspiration - Hypercellular marrow. Megakaryocytes seen. Erythroid hyperplasia. Sea blue histiocytes seen in plenty with occasional hemophagocytes seen. To rule out lipid storage disease. ? Niemann Pick. No evidence of LD bodies.

· Funduscopy – Bilateral mild disc pallor suggestive of Optic atrophy. No cherry red spot.

· Blood sphingomyelinase levels – Normal

· Splenic aspiration for LD bodies – negative

· Ascitic tap – 45 cells (75% polymorphs, 25% lymphocytes), proteins = 0.75 gm %

· Hemoglobin electrophoresis- HbF = 1.6 gm%, HbA2 = 3.1%, HbA = 95.3%.
No evidence of thalessemia or hemolytic anemia

Summary:
A 2-year-old male child born of non-consanguineous marriage from Uttar Pradesh presents with
· Fever since 1 year
· Splenohepatomegaly
· Generalized edema
· Chronic malnutrition
· Generalized lymphadenopathy
· Pancytopenia with hypersplenism
· High ESR
· Hypoalbuminemia
· High alkaline phosphatase
· ? Sea blue histiocytosis on bone marrow aspiration.
· No LD bodies on bone marrow or splenic aspiration
· Bilateral optic disc pallor
· Normal blood sphingomyelinase levels
· HIV negative
· Mantoux test negative

Treatment:
Child was treated with IV Amphotericin B in view of clinical suspicion of Kala Azar but showed no improvement. (Sodium stibogluconate, pentamidine, milfepostine not available). Also required repeated blood transfusion and platelet transfusion in view of pancytopenia. Child has clinically worsened.

HOW TO MANAGE THIS CASE FURTHER? WHAT IS THE DIFFERENTIAL DIAGNOSIS? SHOULD SPLENECTOMY BE DONE IN VIEW OF HYPERSPLENISM?

Answer Discussion :

surender

Other possibilities such as histiocytosis, Kalaazar should be reinvestigated viz. splenic aspirates , skin biopsy of rashes, serology for kalaazar etc.Moreover a possibility of Chronic malaria should be kept and weekly chloroquine for 3-6 months can be empirically started.If pancytopenia or evidence of hypersplenism persists thjen splenectomy after taking precautions can be considered

21 years ago

DR.SHRISHU.R.KAMATH

The question here is to know some more details regarding the case is whether this child had organomegaly -specifically hepatosplenomegaly .This child has pallor and seborrheic dermatitis with generalized edema which is present chronically from the history .hence apossiblity of Protein energy malnutrition-kwashiorkar with underlying lymphoreticular malignancy or chronic infection { TB} or immunodeficiency has to be ruled out. A peripheral smear is of importance as it will help to identify the above conditions. A bone marrow smear and culture will clinch the diagnosis of all above conditions Bone marrow will also help in identifying Histiocytosis and any malignancy.A HIv ELISA should also be done in such cases .

21 years ago

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A 2 year old child with fever, hepatosplenomegaly and generalised lymphadenopathy

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