Congenital Leukemia and Hypoplastic Preleukemia

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CONGENITAL LEUKEMIA & HYPOPLASTIC PRELEUKEMIA

Dr. Bharat R. Agarwal
Pediatric Hematologist-Oncologist, Division of Pediatric Hem-Onco,
B.J. Wadia Hospital for Children

CONGENITAL LEUKEMIA

Leukemia diagnosed from birth to 6 weeks of age is defined as congenital leukemia. This is a rare disease. Its etiology is unknown. Congenital leukemia has been associated with:

Trisomy 7
Turner's syndrome
Mosaic trisomy 9
Mosaic monosomy 7

A few examples of congenital JMML have been reported.

Clinical features

Nodular skin infiltrates (bluish, fibroma-like tumors, leukemia cutis)
Hepatosplenomegaly
Lethargy, poor feeding, pallor
Purpura / petechiae
Respiratory distress

Laboratory studies

Usually monocytic subtype of ANLL.
Occasionally ALL (pre-B immunophenotype)

Management

Congenital leukemia in Down syndrome :
- Therapy should be withheld as long as possible, because spontaneous remission may occasionally occur.
- If disease progresses, hematological or clinical condition deteriorates, appropriate chemotherapy can be administered.
Congenital leukemia with normal blast cell karyotype:
- Therapy should be withheld, because spontaneous remission may occasionally occur.
- If the disease progresses, appropriate therapy can be administered.
Congenital leukemia with chromosomal anomalies in blast cells: This leukemia progresses clinically and hematologically and requires institution of therapy.

Treatment

Treatment involves intensive therapy with VP16 or VM-26. This treatment has been found effective in acute monoblastic leukemia of infancy and neonatal leukemia. Allogenic bone marrow transplantation, if a suitably matched bone marrow donor is available, may be indicated when patient is in remission.

HYPOPLASTIC PRELEUKEMIA

A small number of patients who ultimately develop acute leukemia are initially diagnosed as having aplastic anemia. The illness is characterized by :-

Pancytopenia or single cytopenia
Hypocellular bone marrow
No hepatosplenomegaly
Diagnosis of leukemia 1-9 months after onset of symptoms.

In 1-2% of childhood leukemias, the presentation includes initial marrow failure and may represent 5% of childhood acquired aplastic anemia.

Treatment

Supportive transfusions initially
Specific antileukemic chemotherapy, when leukemia is diagnosed

Last created on 24-06-2002
Last updated on 01-07-2006

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