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1.
The International Medical Journal Malaysia ; (2): 123-126, 2017.
Article in English | WPRIM | ID: wpr-627172

ABSTRACT

Congenital leukemia is an extremely rare disease but frequently fatal. We report a case of intrauterine death (IUD) secondary to congenital erythroid leukaemia associated with maternal Parvovirus B19 infection. Further research is needed to ascertain the association between maternal Parvovirus B19 infection and congenital leukemia.

2.
Article in English | IMSEAR | ID: sea-164825

ABSTRACT

Congenital leukemia (CL) is a very rare disorder. Congenital leukemia is very uncommon myelo proliferative disorder and may be associated with Down syndrome. Congenital Leukemia constitutes approximately 1% of all pediatric leukemias. Though rare, congenital leukemia (CL) is a condition readily recognized by pathologist, hemato-pathologists and pediatricians. Diagnosis can be made by the presence of leukemic cells in peripheral smear and bone marrow. Some cases of congenital leukemias have been reported with skin involvement presenting as reddish pink, light brown or purple papules or nodules and purpura. Congenital leukemia is a rare disorder, but it’s a well-documented disease in which a disease process is detected at birth or very shortly within few days of birth. an estimated 160 to 190 reports of congenital leukemia has appeared in the literature. Most of the cases of CL diagnosed so far by various pathologists and hematopathologist belong to non--lymphoblastic lineage or myeloid lineage. Generally pediatric hematological malignancies are of lymphoid lineage. I have presented here a rare case of congenital leukemia of myeloid lineage which was diagnosed on day one in a low birth weight baby, born to non-consanguineous parents.

3.
Rev. cuba. hematol. inmunol. hemoter ; 31(1): 79-85, ene.-mar. 2015.
Article in Spanish | LILACS | ID: lil-743987

ABSTRACT

La leucemia congénita es una entidad rara que se diagnostica entre el momento del nacimiento y los primeros 30 días de vida. Menos del 1 % de las leucemias de la infancia se diagnostican en el neonato. La hipoplasia severa o aplasia del timo se observa en el síndrome de Di George, que incluye varias malformaciones congénitas y déficit inmunológico, principalmente de células T por hipoplasia del timo, aunque se puede observar aplasia del timo en pacientes sin diagnóstico de Di George. Se presenta un caso diagnosticado como leucemia mieloide aguda congénita. En el momento del nacimiento presentó palidez mucocutánea intensa, petequias, equímosis generalizadas y hepatoesplenomegalia. El diagnóstico se confirmó por la presencia de blastos mieloides en periferia y médula ósea y por la caracterización inmunofenotípica de estas células. La necropsia confirmó la aplasia del timo.


Congenital leukemia is a rare disease in which a leukemic process is presented at birth or during the first 30 days of life. Less than 1 % of childhood leukemia is diagnosed in newborns. The severe hypoplasia or total thymic aplasia is seen at Di George syndrome which includes several birth defects and immune deficit, mainly of T cells by thymic hypoplasia; nevertheless, severe thymic hypoplasia can be observed in patients without diagnosis of Di George. We report a case of congenital acute myeloid leukemia who presented intense paleness generalized petechiae and ecchymoses as well as hepatosplenomegaly. The diagnosis was confirmed by the presence of blasts in peripheral blood smear and bone marrow aspirate. Immunophenotyping was performed and contributed to a definitive diagnosis. The autopsy confirmed the thymic aplasia.


Subject(s)
Humans , Female , Infant, Newborn , DiGeorge Syndrome/mortality , Leukemia, Myeloid, Acute/complications , DiGeorge Syndrome/pathology
4.
Obstetrics & Gynecology Science ; : 325-329, 2014.
Article in English | WPRIM | ID: wpr-37127

ABSTRACT

Congenital leukemia is very rare, and its prevalence according to recently published papers is from 1 to 5 per million live births. This can be often diagnosed in postpartum throughout bone marrow biopsy, showing abnormal proliferation of immature blasts and granulocytic precursors. Hepatosplenomegaly is the most common feature which is found during perinatal examinations, that diagnosing is difficult during perinatal period. Hepatosplenomegaly can occur not only in congenital leukemia but in many other cases such as infection which is the most common cause. In other words, congenital leukemia is the one of the rare causes of hepatosplenomegaly. However, this case shows the fetus with the features of hepatosplenomegaly during perinatal period and being diagnosed as congenital leukemia associated with acquired AML1 gene duplication in postpartum through bone marrow biopsy. Due to its rare instance, we are to describe the case with a review of literatures.


Subject(s)
Biopsy , Bone Marrow , Fetus , Gene Duplication , Leukemia , Live Birth , Postpartum Period , Prevalence
5.
Rev. cuba. hematol. inmunol. hemoter ; 29(4): 318-326, oct.-dic. 2013.
Article in Spanish | LILACS | ID: lil-695869

ABSTRACT

La leucemia congénita constituye una entidad rara que se diagnostica entre el momento del nacimiento y los primeros 30 días de vida. Menos del 1 por ciento de las leucemias de la infancia se diagnostican en el neonato. Se caracteriza por la presencia en un recién nacido, de hepatomegalia, esplenomegalia y lesiones hemorrágicas o infiltrativas en piel. El hemograma completo, el examen de la lámina de la sangre periférica, el aspirado de médula ósea, junto con el inmunofenotipaje y los estudios de biología molecular confirmarán el diagnóstico. El trastorno mieloproliferativo transitorio y las infecciones congénitas constituyen los principales diagnósticos diferenciales. El pronóstico de la enfermedad es sombrío. El tratamiento está basado en regímenes de poliquimioterapia intensiva y el trasplante de progenitores hematopoyéticos constituye una opción terapéutica en un grupo de pacientes


Congenital leukemia is a rare but well documented disease which can be diagnosed on the first 30 days of life. The diagnosis requires a high index of clinical suspicion in a newborn with hepatosplenomegaly and hemorragic or infiltratives cutaneous lesions (red, brown or purple papules or nodules) along with further investigations such as complete blood count, peripheral blood smear, bone marrow aspirate and immunopfenotypic profile. A true congenital leukemia has to be distinguished from other conditions such as a transient mieloproliferative disorder described in Down´s syndrome and congenital infections. The prognosis of neonatal leukemia is dismal. Treatment includes intensive multi-agent chemotherapy and supportive care, being the hematopoietic stem cell transplantation an additional treatment option in a group of patients


Subject(s)
Humans , Male , Female , Infant, Newborn , Leukemia/complications , Leukemia/congenital , Leukemia/physiopathology
6.
Article in English | IMSEAR | ID: sea-167413

ABSTRACT

Congenital leukemia is a rare disorder presenting in the immediate neonatal period & diagnosed at or within few days of birth.1 Because of the doubling time of leukemic cells, the disease becomes clinically evident after birth or shortly thereafter.2 Neonates with Down’s syndrome have an increased risk for congenital leukemia particularly acute megakaryoblastic leukemia (FAB M7).3 The incidence of acute megakaryoblastic leukemia in Down’s syndrome is estimated to be 400 times than in normal children.4 We present a case of congenital leukemia in a 22 day old female child with Down’s syndrome who had cyanosis and skin rashes for 2 days. Diagnosis was established on the basis of hematological investigations and cytochemistry of the cells

7.
Rev. cuba. pediatr ; 83(2): 193-199, abr.-jun. 2011.
Article in Spanish | LILACS | ID: lil-615686

ABSTRACT

La leucemia aguda durante el período neonatal es poco frecuente de evolución rápida y pronóstico sombrío. Sus características clínicas y biológicas difieren de las encontradas en niños de mayor edad, y su inicio se caracteriza por afectación cutánea, hepatoesplenomegalia, hiperleucocitosis e infiltración del sistema nervioso central. Se han observado pacientes con formas tanto mieloides como linfoides, pero la leucemia mieloide aguda parece predominar en esta etapa de la vida. Se presenta el caso de un paciente con leucemia congénita clasificada morfológicamente, con aparición de manifestaciones clínicas de enfermedad hematológica desde el nacimiento y diagnóstico de leucemia linfoblástica aguda congénita


Acute leukemia during neonatal period is not frequent, of a fast course and gloomy prognosis. Its clinical and biological features differ of that present in older children and it onset is characterized by cutaneous affection, hepatosplenomegaly, hyperleukocytosis and infiltration of central nervous system (CNS). There are patients presenting with myeloid and lymphoid types, but the acute leukemia seems to predominate in this stage of life. This is the case of a patient with acute leukemia morphologically classified, with appearance of clinical manifestations of hematologic disease from birth and a diagnosis of congenital acute lymphoblastic leukemia

8.
Article in English | IMSEAR | ID: sea-171949

ABSTRACT

Congenital leukemia is a rare disease that can manifest soon after birth. Cutaneous involvement consists of red, brown or purple papules or nodules and purpura. We present a case of congenital myelomonblastic leukemia in a seven week old infant who had petechiae and subcutaneous nodules. Diagnosis was established by the presence of leukemic cells in bone marrow and involved skin along with cytochemical characterization of these cells.

9.
Annals of Dermatology ; : 66-70, 2009.
Article in English | WPRIM | ID: wpr-45340

ABSTRACT

Congenital leukemia is a rare disease that develops from birth to 6 weeks of life. Leukemia cutis involves cutaneous infiltration by leukemic cells and is an unusual manifestation of leukemia, and has been documented in 25~30% of patients with congenital leukemia. The authors report a case of congenital leukemia cutis. A newborn male presented with widespread firm dusky red papules and nodules on almost his entire body surface. Skin biopsy specimens confirmed the presence of leukemic infiltrations, and bone marrow cytology was consistent with acute myeloid leukemia of the FAB M5 type.


Subject(s)
Humans , Infant, Newborn , Male , Biopsy , Bone Marrow , Leukemia , Leukemia, Myeloid, Acute , Leukemic Infiltration , Parturition , Rare Diseases , Skin
10.
Annals of Dermatology ; : 74-76, 2008.
Article in English | WPRIM | ID: wpr-171037

ABSTRACT

Congenital leukemia is a rare condition diagnosed at birth to 6 weeks of life with an estimated incidence of 4.7 per million live births. In a review, about 25~30% of cases were well documented as leukemia cutis. We report a case of a 3-month-old infant presented with asymptomatic multiple erythematous to bluish patches and nodules, which had developed about two months ago. Biopsy of the cutaneous lesions revealed immature cells which are overall monotonous with large kidney shaped nucleus, prominent nucleoli and moderate cytoplasm. These atypical cells stained positive for leukocyte common antigen, lysozyme and CD68. With this result, a congenital leukemia cutis was diagnosed. Six months later, she underwent cord blood stem cell transplantation. Seven months after the cord blood stem cell transplantation, the bone marrow examination revealed a normal result. Leukemia can be presented in the skin, the eruption may be nonspecific and it may precede systemic involvement. The recognition is important because early diagnosis can lead to appropriate treatment, followed by better prognosis.


Subject(s)
Humans , Infant , Leukocyte Common Antigens , Biopsy , Bone Marrow Examination , Cord Blood Stem Cell Transplantation , Cytoplasm , Early Diagnosis , Incidence , Kidney , Leukemia , Live Birth , Muramidase , Parturition , Prognosis , Skin
11.
Korean Journal of Dermatology ; : 1089-1093, 2000.
Article in Korean | WPRIM | ID: wpr-12888

ABSTRACT

Congenital leukemia is a rare disease developing within the first 4 to 6 weeks of life, whose natural history is quite different from that of childhood and adult leukemia. Leukemia cutis occurs in 25% to 30% of infants with congenital leukemia and is more frequently associated with acute myeloid leukemia than acute lymphocytic leukemia. We report a case of congenital leukemia cutis in which a walnut-sized, slightly erythematous, dome-shaped tumor that had developed at left frontal area since birth was confirmed by skin biopsy. A review and analysis of Korean literature pertaining specially to leukemia cutis in congenital leukemia is presented along with the report of an additional case.


Subject(s)
Adult , Humans , Infant , Biopsy , Leukemia , Leukemia, Myeloid, Acute , Natural History , Parturition , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Rare Diseases , Skin
12.
Korean Journal of Clinical Pathology ; : 392-395, 1999.
Article in Korean | WPRIM | ID: wpr-41947

ABSTRACT

The patient was a 1-day-old female with full term normal vaginal delivery. Petechiae and purpura were noted on body surface at birth. She did not have mongoloid face and hand-foot deformity. The skin nodules were bluish to slate-grey in color, and observed on all body surfaces. Hepatosplenomegaly was also noticed. Peripheral blood smear showed marked leukocytosis (109,400/microliter), thrombocytopenia (16,000/microliter) and leukoerythroblastosis. VDRL and TORCH tests were negative. The bone marrow was replaced by leukemic lymphoblasts. The normal hematopoietic cells were depleted. The leukemic lymphoblasts expressed markers of mature B cell differentiation, negative for Sudan black B, peroxidase, and positive for PAS (en bloc pattern) stain. Skin biopsy specimen showed diffuse infiltration of the leukemic cells. The diagnosis was congenital mature B cell lymphoblastic leukemia. This patient died at 7 days after birth.


Subject(s)
Female , Humans , Biopsy , Bone Marrow , Cell Differentiation , Congenital Abnormalities , Diagnosis , Leukocytosis , Parturition , Peroxidase , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Purpura , Skin , Sudan , Thrombocytopenia
13.
Korean Journal of Pathology ; : 308-316, 1988.
Article in Korean | WPRIM | ID: wpr-131514

ABSTRACT

Leukemia is a rare disease in the newborn infant. We have presented an autopsy case of congenital acute myelocytic leukemia in a female neonate and discussecd with review of literature. At birth, she was relatively in good health with 4.2 kg in body weight except a large cephalhematoma on left parietal scalp and multiple subcutaneous nodules with ecchymosis on entire body surface. Hemoglobin concentration was 12.0 gm/, Hct 34.6% and erythrocyte count was 2.24 millions. Of 212,400 leukocytes/mm2, 47% were myeloblast. Biopsy of skin nodules reveal leukemia cutis, which disappear dramatically with anticancer drug. The infant was expired 12 days after admission due to intracerebral hemorrhage and acute renal failure.


Subject(s)
Infant , Male , Female , Infant, Newborn , Humans , Biopsy
14.
Korean Journal of Pathology ; : 308-316, 1988.
Article in Korean | WPRIM | ID: wpr-131511

ABSTRACT

Leukemia is a rare disease in the newborn infant. We have presented an autopsy case of congenital acute myelocytic leukemia in a female neonate and discussecd with review of literature. At birth, she was relatively in good health with 4.2 kg in body weight except a large cephalhematoma on left parietal scalp and multiple subcutaneous nodules with ecchymosis on entire body surface. Hemoglobin concentration was 12.0 gm/, Hct 34.6% and erythrocyte count was 2.24 millions. Of 212,400 leukocytes/mm2, 47% were myeloblast. Biopsy of skin nodules reveal leukemia cutis, which disappear dramatically with anticancer drug. The infant was expired 12 days after admission due to intracerebral hemorrhage and acute renal failure.


Subject(s)
Infant , Male , Female , Infant, Newborn , Humans , Biopsy
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