Leukemia cutis in an infant with congenital leukemia and tetralogy of fallot.

Congenital leukemia is a very rare severe condition and leukemia cutis may represent the presenting sign of this malignancy, sometimes preceding hematological findings of weeks. Typical clinical features include multiple red to purple papules, macules and nodules due to direct infiltration of the skin by malignant cells. We illustrate these cutaneous findings in a patient with congenital leukemia and tetralogy of Fallot.

INTRODUCTION

Congenital leukemia is a very rare severe condition and leukemia cutis may represent the presenting sign of this malignancy, sometimes preceding hematological findings of weeks.[1]

Typical clinical features include multiple red to purple papules, macules and nodules due to direct infiltration of the skin by malignant cells. We illustrate these cutaneous findings in a patient with congenital leukemia and tetralogy of Fallot.

A term male infant born to unrelated Bengali parents by emergency cesarean section for fetal bradycardia, after a not followed pregnancy, showed since birth peculiar widespread skin lesions. Bluish macules, not disappearing under pressure and purple nodules (about 0.5 cm diameter) were present over the majority of the body surface including head, lips and chin [Figure 1]. The baby presented a mild respiratory distress with oxygen requirement in delivery room, hepatomegaly, a systolic murmur and no dysmorphic features. Maternal serology, including TORCH, hepatitis B surface antigen, hepatitis C virus, venereal disease research laboratory and treponema pallidum hemagglutination assay, was negative. Complete blood count on admission showed hyperleukocytosis (white blood cell 152 × 103/μl with presence of all the immature forms of the granulocytic series, from promyelocytes to metamyelocytes and 5% of blasts; red blood cell 3.77 × 106/μL; hemoglobin 11.5 g/dL; hematocrit 33%; platelets 234 × 103/μL). Coagulation tests were altered. Other routine blood tests, including inflammatory markers, were normal. Cranial ultrasound scan was normal. Echocardiography showed the presence of a tetralogy of Fallot. Bone marrow examination confirmed the diagnosis of acute myeloid leukemia.

Figure 1

(a) Cutaneous lesions present since birth over the majority of the body, as described in the text. (b) Macules and nodules overhead, lips and chin

The association of congenital leukemia and tetralogy of Fallot was previously described only in one patient with Down's syndrome.[2]