The patient was admitted to oncohematology department on 02.04.2009 with complaints for sleep disturbance, decrease of appetite, and recurrent fever for 2 months. According to her parents, the mentioned complaints appeared following vaccination in the age of 3 months. The disease anamnesis signifies that the patient was born mature following pregnancy of pathological course (toxicosis for 8 months) with the weight 2750 g, and discharged from the hospital on the 3-rd day after birth. The patient has been BCG vaccinated. The data confirming prenatal infection are absent in the extract issued by maternity hospital.

The objective condition during admission to the hospital was severe due to intoxication and anemic syndrome, fever, hepatosplenomegaly, peripheral lymph nodes of polyadenopathy type, and punctuated papular rash on crus skin.

Blood count of 02.04: L-39 x 10⁹/l (neutrophils – 21%, stab neutrophils – 7%, metamyelocytes – 2%, myelocytes – 4%, promyelocytes – 1%, blasts – 2%, lymphocytes – 46%, monocytes – 13%, basophils – 1%), Hb 77 g/l, platelets – 115 x 10⁹/l; blood count of 14.04: L-41.9 x 10⁹/l (neutrophils – 26%, stab neutrophils – 7%, metamyelocytes – 5%, myelocytes – 6%, blasts – 3%, monocytes – 14%, lymphocytes – 39%), platelets – 109 x 10⁹/l Hb 71 g/l. Fetal hemoglobin – 0.67%.

Biochemical blood count: protein 66 g/l, ALT (alanine transaminase) 27 U/L, bilirubin – 13.3 µmol/l, urea – 2.5 mmol/l, serum iron – 12.5 µmol/l, triglycerides – 2.47 mmol/l, glucose – 4.2 mmol/l.

Myelogram data of 02.04.2009: blasts 10.2% (a copy and the slides are added). Cytogenetic study of bone marrow revealed no clonal abnormalities.

RW test result is negative.

The patient has been consulted by:

-         infectious disease specialist – the data confirming prenatal infection of CMV, toxoplasmosis are absent (the data of serologic examinations are added);

-         geneticist – storage diseases (Gaucher's disease, Niemann-Pick disease) are excluded;

-         ophthalmologist – ocular media reveal no pathologies.

 

On the basis of the aggregate of data, including fever without response to antibacterial therapy performed for 3 months, hepatosplenomegaly (liver, lien at navel level), polyadenopathy, anemic syndrome, rash on crus skin, peripheral blood leucocytosis > 10,000/µl with the presence of myeloid precursors, monocytosis exceeding 1000/µl, blast level on myelogram <20%, and the absence of Ph chromosome, the child was diagnosed with

The only mode of therapy is allogenic transplantation of bone marrow. As there is no sibling in the family, allogenic transplantation from an alternative donor is indicated.

14.04.2009