Congenital Leukemia & Hypoplastic Preleukemia

Bharat R Agarwal
Pediatric Hematologist-Oncologist, Division of Pediatric Hem-Onco, B.J. Wadia Hospital for Children, Mumbai, India.
First Created: 01/04/2001 

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.

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 the onset of symptoms.

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

Clinical Features - Congenital Leukemia

  • Nodular skin infiltrates (bluish, fibroma-like tumors, leukemia cutis)

  • Hepatosplenomegaly

  • Lethargy, poor feeding, pallor

  • Purpura/petechiae

  • Respiratory distress

Congenital Leukemia - Laboratory Studies

  • Usually monocytic subtype of ANLL.

  • Occasionally ALL (pre-B immunophenotype)

Congenital Leukemia - Management

Congenital leukemia in Down syndrome:
Therapy should be withheld as long as possible, because spontaneous remission may occasionally occur. If the disease progresses, the 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 the institution of therapy.

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 the patient is in remission.

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 the patient is in remission.

Treatment

Supportive transfusions initially. Specific antileukemic chemotherapy, when leukemia is diagnosed.


Congenital Leukemia & Hypoplastic Preleukemia Congenital Leukemia & Hypoplastic Preleukemia Congenital Leukemia & Hypoplastic Preleukemia 01/04/2001
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