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We here describe a case of congenital leukemia that ended in intrauterine fetal demise at 30 weeks of gestation. Acute enlargement of the fetal trunk, elevated pulsatility index of the umbilical artery with concomitant decline of pulsatility index of the middle cerebral artery, pleural effusion, and polyhydramnios preceded the fetal death. Diagnosis of congenital myeloid leukemia was suggested by microscopic examination of the placental tissue, revealing immature myeloid precursors filling the lumina of fetal vessels in the umbilical cord and chorionic villi. Extensive vascular involvement of the placenta by leukemic cells was considered to be a primary cause of the fetal death., (Copyright © 2011 S. Karger AG, Basel.)

Congenital leukemia is seldomly diagnosed. Cases should be differentiated from transient leukemoid reaction, which is noted in Down syndrome. Outcome in congenital leukemia is poor, but spontaneous remissions have been described. The authors report on a female neonate with myeloid leukemia of the skin; no blood and bone marrow involvement was noted. Constitutional 47, XX, + 21 was excluded. In situ hybridization on a paraffin-embedded skin biopsy sample did not show trisomy 21 in the leukemia lesions. No antileukemia therapy was given. During follow-up, small nodules (diameter up to 3 mm) on the soles of both feet came and went over a 3-month period. The child is now 3.5 years old and well. To date, 18 cases of congenital leukemia showing spontaneous remission have been described in the literature, almost exclusively myeloid leukemia (FAB M4 and M5). Congenital leukemia confined to the skin was described in only 4 cases. On follow-up, 6 cases relapsed; only one of them initially had skin involvement only. The data from this patient and literature indicate that cytostatic treatment should start only if the malignancy interferes with vital parameters. In case of relapse or progression, initial postponement of chemotherapy in these frail neonates will result in less toxicity and probably a better survival.

Among the various anomalies associated with Down syndrome, leukemia is quite common. The variant transient myeloid leukemia is seen almost exclusively in the Down syndrome patients. On the other hand, urological anomalies are infrequently found both in the Down syndrome and leukemia patients. We report a case who had the rare combination of a urological anomaly along with Down syndrome and transient myeloid leukemia.

Anewborn with a transient myeloproliferative disorder and a myeloid/natural killer cell leukemia phenotype is described. The blasts expressed CD7, CD33, CD34, CD56, and CD117 but did not react with cytoplasmic myeloperoxidase and were negative for cy CD22, HLA-DR, and CD90 expression. No megakaryoblastic surface markers were identified. The blast population disappeared from the peripheral blood and bone marrow within 2 months, but hepatomegaly and recurrent respiratory insufficiency persisted. The patient died of unilateral pneumonia in the third month of life. Neither extramedullary infiltration nor other hematologic signs of disease progression were found.

We reviewed Dutch patients and those described in the literature with congenital leukaemia in the past 25 years, with the intention to obtain an overview of the characteristics of this rare disease. Among the 117 patients reviewed, acute myeloid leukaemia (AML) was more frequent (64%) than acute lymphoblastic leukaemia (ALL, 21%). Most patients had a high leukaemic cell load with hepatosplenomegaly, leukaemia cutis and hyperleucocytosis. Cytogenetic abnormalities were found in the majority of the patients tested (72%); 11q23 abnormalities were found in less than half of them (42%). The probability of overall survival at 24 months was only 23%. When congenital AML and ALL were compared, clinical characteristics and overall survival were not significantly different. However, in patients at risk, the probability of event-free survival (EFS) and disease-free survival (DFS) were significantly higher in AML than in ALL, 43% versus 13% and 68% versus 0% respectively. Among the congenital AML cases, six spontaneous remissions have been described. In conclusion, the clinical characteristics of congenital leukaemia differ from those of leukaemia in older children and prognosis is generally poor. Once complete remission is achieved, patients with AML fare better than those with ALL. Chemotherapy for congenital leukaemia needs improvement to increase the sustained remission rate.

Congenital leukaemia (CL) is a rare disorder that presents with extramedullary infiltrates and a myeloid phenotype. CL can progress rapidly without adequate treatment, but, paradoxically, may also remit spontaneously. Because of the significant toxicity involved in delivering chemotherapy to newborns, it is important to identify those newborns who may not require treatment. We describe an infant who presented at 1 week of age with congenital myeloid leukaemia. Cytogenetic analysis revealed a t(8;16)(q11;p13) translocation. The infant's leukaemia underwent a spontaneous regression. This case further confirms the possibility of spontaneous remission in congenital leukaemia. Moreover, it suggests that the presence of a clonal cytogenetic aberration does not preclude the possibility of a spontaneous regression in CL.

A newborn infant with spontaneous remission of congenital leukemia cutis is described and a literature review of this uncommon phenomenon is provided. In view of the unusual and unpredictable behavior of this disease, chemotherapy should be withheld unless there is evidence of an 11q23 translocation or progressive disease. Otherwise, overall survival does not appear to be affected by adopting a conservative approach. Because of occasional late relapses, long-term follow-up is recommended. The biologic basis underlying spontaneous remission of congenital leukemia is unknown; therefore, molecular or molecular cytogenetic analysis of DNA obtained from a skin biopsy is recommended.

We report an unusual case of congenital leukemia with leukemia cutis (LC) and diffuse calcinosis cutis. A newborn girl presented with widespread dusky red and yellowish cutaneous nodules and papules. Bone marrow morphology was consistent with the diagnosis of acute monocytic leukemia of the FAB M5 type. Skin biopsy specimens confirmed the presence of a leukemic infiltrate and revealed calcium salt deposition in the papillary and reticular dermis. Calcinosis was diffuse in the whole skin but spared other organs. Vascular calcification was not present. Serum calcium levels oscillated between 2.5 and 2.86 mmol/l, and phosphorus, parathyroid hormone and 25-hydroxyvitamin D(3) levels were normal. There were diffuse osteoporosis and spontaneous fractures of small tubular bones. The patient responded to chemotherapy but, following consolidation treatment, developed sepsis and died at 120 days of age. Congenital leukemia is rare and LC is uncommon. Hypercalcemia may be a complication of leukemia, which leads to multiorgan metastatic calcification. Despite the absence of frank hypercalcemia, the presence of bone lesions suggests that the patient's calcinosis cutis was of the metastatic type. However, the cutaneous leukemic infiltrate may also represent a triggering factor for calcium deposition in the skin., (Copyright 2000 S. Karger AG, Basel.)

Most patients with congenital leukemia do not survive past infancy despite aggressive chemotherapy. We describe three patients with congenital leukemia who have undergone prolonged periods of spontaneous remission. Our experience suggests that some patients with congenital leukemia may benefit from initial conservative management without chemotherapy. We summarize the clinical presentations of these patients and review the literature.

We present the case of a 4-day-old boy with acute lymphoblastic leukemia showing at onset a karyotype 46,XY,t(4;11)(q21;q23). At relapse an additional change, add(2), was present. Molecular analysis showed the same immunoglobulin rearrangement both at onset and at relapse, but immunohistochemical analysis revealed some cells having myeloid features. A continuous cell line derived from the leukemic blasts of the patient presented typical monoblastic features.

The case of a patient with ecchymosis, hepatomegaly, leukocytosis, thrombocytopenia, and anemia at birth is presented. Throughout his course, thrombocytopenia, anemia, and leukocytosis without a marked increase in the number of blast forms in either peripheral blood or bone marrow persisted until the patient developed a blast crisis shortly before his death at age 4 months. This patient is the youngest reported to have the juvenile form of chronic myelogenous leukemia and the first that in the present era can be considered congenital in origin.

Two cases of infants with congenital leukemia who had severe, refractory hypertensive reactions to teniposide (VM-26) are described. Patients on a 5 mg/kg twice weekly schedule of teniposide had hypertensive reactions in which their systolic blood pressure was greater than 200 mm Hg after the second dose of teniposide. Hypertension combined with myelosuppression resulted in the patient's death in one case. Although the exact mechanism of this unusual toxicity of teniposide remains unknown, it might be an age-specific problem, considering the very young age of our patients. Meticulous monitoring of vital signs, including blood pressure, is mandatory in leukemic infants receiving teniposide.

A case of congenital leukemia that originally did not express any lineage-specific antigenic markers is presented. The blast cell morphologic appearance was L1 according to French-American-British (FAB) classification and showed lymphoid characteristics by cytochemical staining and transmission electron microscopy. However, immunophenotyping using a variety of monoclonal antibodies did not confirm the lymphoid origin. Furthermore, the immunoglobulin heavy chain and T-cell receptor beta-chain genes were in germ-line configuration. The in vitro culture study defined the leukemia as of myeloid origin. The semisolid methylcellulose culture showed an acute non-lymphocytic leukemia-type growth pattern. Bone marrow blasts underwent myeloid differentiation with positive myeloperoxidase and butyrate esterase activity during a suspension culture. These findings indicate that this case represents an acute undifferentiated leukemia that has probably arisen from the malignant transformation of stem cells of myeloid progeny.