What is Congenital Leukemia
Congenital leukemia (congenital leukemia, CL) refers to the first six weeks after birth from birth to diagnosis of leukemia. 6 weeks to 6 months after birth, called neonatal leukemia. CL is a rare disease, the incidence rate is about surviving infant 4.7 / million, accounting for 1/4 of the innate malignancy, second only to neuroblastoma, ranking the first two, the prognosis is poor, and mortality of 92.3%.
Symptoms of Congenital Leukemia
Clinical manifestations of congenital leukemia:
Approximately 50% of patients with congenital leukemia leukemia, skin nodules, about 0.2 ~ 0.3cm size, was the schungite or fuchsia or normal, palpable deep skin fibers, such as tumor-like mass, no adhesions, distributed throughout the body, infiltrating cells The myeloid few lymphocytes and monocytes Department. Followed by papules, erythema multiforme, eczema or herpes-like lesions. Purpura, umbilical cord, gastrointestinal tract, urogenital tract, and intracranial hemorrhage. Liver splenomegaly, lymphadenopathy rare. Tissues and organs outside the hematopoietic tissue extensive involvement of the central nervous system and testes to leukemia early stage. Rapid progression, short course, and poor response to chemotherapy, and more short-term (usually 1 week to several months) deaths. Fetal hydrops and stillbirth in the fetal period, mild to moderate edema huge placenta, tumor cells are widely visceral infiltration, occurs in 34 to 38 weeks of pregnancy.
Peripheral blood leukocytes Ming significantly increased, up to 15 x 10 ^ 9 / L (2 to 85 x 10 ^ 9 / L), dominated the original cell. Hemoglobin 30 ~ 120g / L, teardrop and nucleated red blood cells. Platelet generally <70 × 10 ^ 9 / L (60 to 300 × 10 ^ 9 / L). Hyperactive bone marrow hyperplasia, plain undifferentiated cells, significantly reduced red blood cell system, megakaryocytes and mature granulocytes. ALL previous B-cell majority. Common chromosomal abnormality t (4; 11) (q21; q23), accounting for ALL the 78%, the ANLL's 10%; t (4; 11), t (1; 4) and IgHcJ gene amplification rearrangement of prognosis difference, t (11; 9) the prognosis is good.
What Causes Congenital Leukemia
The etiology and pathogenesis of congenital leukemia is still not clear, and may be associated with genetic and physical abnormalities. Often accompanied by congenital malformations, such as 21 - trisomy, Tumer syndrome - trisomy 13 - trisomy. CL to AML (about 46% to 89%), followed by In ALL, M5, MUL and M6 JCML also reported.
Tests and Diagnosis for Congenital Leukemia
Congenital leukemia diagnostic tests:
Diagnostic criteria: ① The blood and bone marrow myeloid or lymphoid undifferentiated cells or immature cells; ② non-hematopoietic organs corresponding cell infiltration; leukemoid reaction (3) except for cause factors.
The differential diagnosis of congenital leukemia:
Neonatal period there are a variety of diseases that can be confused with the following diseases should pay attention to.
(1) with Down syndrome, myeloproliferative disorder (transient leukemia-like reaction): clinical and hematological congenital leukemia difficult to distinguish between peripheral original grain up to 0.95, the bone marrow of the original tablets from 0.1 to 0.60 . Return to normal within a few weeks to a few months. The autopsy especially Leukemia evidence, in vitro bone marrow CFU-GM sets off the normal chromosome 21 - trisomy chimera.
(2) the type of leukemia reaction: congenital infections such as syphilis, cytomegalovirus inclusion disease, rubella and toxoplasmosis, neonatal bacterial infection and neonatal hemolytic disease can star class leukemia reaction.
(3) neuroblastoma: liver, the subcutaneous nodules like congenital leukemia and bone marrow involvement. However, the primary site of tumor, the the urinary VMA increase in bone marrow smears can be found in neuroblastoma cells can be different.
Treatments of Congenital Leukemia
Congenital leukemia treatment:
Down syndrome and the possible congenital leukemia and normal karyotype CL naive white hair alleviate, as much as possible where he died or observation. The progression of the disease or abnormal karyotype CL AL chemotherapy. VP16 or VM26 M5. CR as possible after hematopoietic stem cell transplantation.