A.K.Jaleel Ahamed, K.Raghu & .A.Subas Mohan Doss
Sri Ramakrishna Hospital,Coimbatore, Tamil Nadu ,641044

Term AGA male baby, 2nd sibling of non consanguineous marriage delivered by emergency LSCS for oligohydramnios was admitted in NICU for respiratory distress & massive hepatosplenomegaly. Antenatal history uneventful. Workup & evaluation was done. Baby was treated with Nasal CPAP, IV fluids and IV antibiotics. At 33 hours of life baby developed hypercatabolic state, hyperkalemia, desaturation & bradycardia which necessitated ventilator support, ionotropes, peritoneal dialysis and 2 units of blood. Peripheral smear revealed a diagnosis of congenital leukemia. Further workup revealed association with Downs ‘Syndrome and immunophenotyping confirmed as AML.

Hemoglobin -13.3gm/dl, Platelet count -2,07,000/cumm, WBC count -1,18,200/cumm

• Random Blood Sugar-22mg%,
• Immunophenotyping – Morphology & FCM phenotyping diagnostic of AML- M5a, M-5a (FAB)
• Karyotype- 47XY, +21, Down's syndrome (Pure trisomy 21).

• Uric acid-12.5mg% , LDH-60u/l, Calcium-8.6mg%, Alkaline Phossphatase-2821u/l
• Total bilirubin-6.8mg%, (Direct -1.0mg%, Indirect-5.8mg%)
• Total protein-6.2gms%, Albumin-3.5gms%, Globulin-2.7gms%, A/G ratio-1.2:1
• SGPT-386U/l
• CRP-Positive
• Urea-88mg%, Creatinine-1.4mg%,
• Serum sodium-123Meq/l, potassium-9.6Meq/l , chloride-100Meq/l, HCO3-5.8Meq/l,

• Echocardiography- Primary pulmonary hypertension, PA pressure 34mm/hg, PDA 2mm with Left to right shunt, No congestive heart failure.
• Cranial ultrasound - Normal scan,
• Blood culture- No growth,

In view of poor prognosis and low survival among patients with neonatal leukemia the parents took the baby against medical advice on 1-6-2005 .

This case was presented for its rarity. No more than 150 cases of neonatal leukemia had been reported in the literature. Congenital leukemia is defined as leukemia that develops in utero. Because of the doubling time of leukemic cells, the disease should be clinically evident within the first month of life . Thereafter, the terms perinatal or infantile leukemia may be used. In contrast to childhood leukemia in which acute lymphoblastic leukemia predominates, in congenital leukemia, acute myelocytic leukemia outnumbers acute lymphoblastic leukemia Though more than 100 well documented cases have been described , the exact incidence of congenital leukemia is unknown. Of the cases reported as congenital leukemia, a majority had acute myeloid leukemia [AML]. The etiology of congenital leukemia like that of other leukemia is unknown. Congenital leukemia has occurred in infants whose mother had leukemia, before or during pregnancy. Fetal x ray exposure has not consistently been associated with an increased incidence of leukemia. Association with chromosomal anomalies like Down's syndrome, Turner's syndrome mosaic monosomy and trisomy has been well documented. Signs and symptoms in the newborn are petechiae, purpura, hepatosplenomegaly, lethargy, pallor and poor feeding. Many infants die of respiratory distress secondary to pulmonary leukostasis and Bronchopneumonia.

Last updated: 1-12-2006

Jaleel Ahamed AK, Raghu K, Doss ASM. Neonate with Fatal Hematological Disorder. Pediatric Oncall [serial online] 2006 [cited 2006 December 1];3. Available from:
http://www.pediatriconcall.com/fordoctor/viewersChoice/
neonatal_fatal_hematological_disorder.asp