Spontaneous Remission Of Congenital Acute Nonlymphoblastic Leukemia With Normal Karyotype In Twins
Mora, J., Dobrenis, A. M., Bussel, J. B., & Aledo, A. (2000). spontaneous remission of congenital acute nonlymphoblastic leukemia with normal karyotype in twins. Medical and pediatric oncology, 35(2), 110–113. https://doi.org/10.1002/1096-911x(200008)35:2<110::aid-mpo4>3.0.co;2-z
View Original Source →Abstract
BACKGROUND: Congenital acute nonlymphoblastic leukemia (cANLL) is an extremely rare event and represents only 0.5-1% of the leukemias in the first year of life. It is usually more common among patients with chromosomal abnormalities. Transient myeloproliferative disease (TMD) is an hyperleukocytosis entity that occurs almost exclusively in Down syndrome patients and remits spontaneously. Spontaneous remission of congenital leukemia has been reported and related to the presence of an extra chromosome 21. PROCEDURE: A pair of non-Down syndrome newborn twins presented with a clinical picture of skin rash and hyperleukocytosis. Twin B had full-blown cANLL with bone marrow, peripheral blood, skin, CSF, and placental invasion. Twin A presented transient peripheral blood and skin involvement by the same type of blast cells. No cytotoxic therapy was given. With 2 years follow-up, they continue to do well. RESULTS: Histologic and immunophenotypical analysis of placentas, cord blood, skin, CSF, bone marrows, and peripheral blood revealed a consistent picture of intrautero cANLL in twin B, with transplacental invasion of twin A. Normal and blast cells were found to be karyotypically normal. Spontaneous remission occurred. CONCLUSIONS: cANLL with karyotypically normal blasts can develop a self-limited clinical course, which has resemblances to TMD.
Case Details
Disease Location
Skin
Personal Characteristics
Twin b large numbers of immature mononuclear cells were present in chorionic and fetal stem blood vessels of the placenta placentas were fused and mature, with two chorions and two amniotic sacs
Clinical Characteristics
Initial physical noted multiple blueish nodules "blueberry muffin spots" on the trunk and forehead liver was enlarged to 3 cm below the right costal margin and the spleen tip was palpable at 24 hours of life, wbc was at 106,000/mm^3 and 70% immature monoblasts, hgb was 14.5g/dl, and platelet count was 104,000/mm^3 immunophenotypic analysis of the peripheral blood demonstrated a preponderance of myeloid cells cd11c, HLA-dr, and my8, a majority expressed CD14, and 13 acute congenital myeloid leukemia of monocytic lineage m5 was suggested lumbar puncture showed similar-appearing cells in the csf but fewer than 5 cells/hpf marrow aspirate performed on day 3 showed virtual replacement by immature monocytoid cells with folded nuclei and scanty cytoplasm, which were strongly positive for alpha naphthyl butyrate skin biopsy performed at day 4 demonstrated a monomorphous myelomonocytic infiltrate of the dermis and subcutaneous tissue consistent with leukemia cutis was diagnosed as congenital call acute non lymphoblastic leukemia (call) treatment included hydration and allopurinol thrombocytopenia persisted and neutropenia subsequently developed one red cell transfusion was administered because hgb was less than 8g/dl immunophenotypic analysis of the marrow biopsy on day 14 of life indicated virtual absence of lymphoid cells, the majority of cells were of myeloid lineage as determined by lysozyme, cd11c, CD14 and 15, a majority showed CD68 reactivity she had one generalized seizure during hospitalization
Remission Characteristics
The 7 following days with treatment, the skin lesions resolved completely and the wbc was normal for age by day 10 of life repeat bone marrow exam on day 14 of life showed evidence of multilineage maturation, and there was a leftward shift of the myeloid lineage, only rare mature forms were identified lymphocytes decreased also on day 14, repeat lumbar puncture was negative for abnormal cells (ccg csf-1) the skin lesions spontaneously disappeared over the course of 2 weeks she was discharged at 17 days of life with persistent liver enlargement and borderline neutropenia at subsequent follow-up, her hepatomegaly had resolved apart from a solitary cafe-au-lait spot on the abdomen, physical was unremarkable, cbc was normal
Treatment & Mechanisms
Proposed Remission Mechanisms
The invasiveness of these karyotypically normal blasts was self-limited which resulted sr
Clinical Treatment
Hydration and allopurinol rbc transfusion
Non-Clinical Treatment
None reported