Biomaterial Database

Transient abnormal myelopoiesis associated with Down syndrome presenting as severe hydrops fetalis: a case report. 2010

G L Malin, and Mark D Kilby, and M Velangi

School of Clinical and Experimental Medicine, University of Birmingham, Birmingham Women's Foundation Trust, Edgbaston, Birmingham, UK.

We present a case of transient abnormal myelopoiesis (TAM) presenting as non-immune fetal hydrops (NIHF). Hydrops fetalis (HF) is a condition associated with very high perinatal mortality, especially when no treatable cause, such as fetal anaemia, exists. In fetuses prior to 24 weeks with NIHF, a chromosomal anomaly is a common association. TAM is a leukaemic condition, almost entirely limited to children with Down syndrome. The presentation of TAM prenatally is unusual but cases may present ultrasonographically with NIHF and associated fetal hepatosplenomegaly. We report a case presenting in this manner with NIHF detected at 29 weeks' gestation and discuss the subsequent diagnosis and management of in utero TAM.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D011247	Pregnancy	The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.	Gestation,Pregnancies
D011263	Pregnancy Trimester, Third	The last third of a human PREGNANCY, from the beginning of the 29th through the 42nd completed week (197 to 294 days) of gestation.	Pregnancy, Third Trimester,Trimester, Third,Last Trimester,Last Trimesters,Pregnancies, Third Trimester,Pregnancy Trimesters, Third,Third Pregnancy Trimester,Third Pregnancy Trimesters,Third Trimester,Third Trimester Pregnancies,Third Trimester Pregnancy,Third Trimesters,Trimester, Last,Trimesters, Last,Trimesters, Third
D004314	Down Syndrome	A chromosome disorder associated either with an extra CHROMOSOME 21 or an effective TRISOMY for chromosome 21. Clinical manifestations include HYPOTONIA, short stature, BRACHYCEPHALY, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, single transverse palmar crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213)	Mongolism,Trisomy 21,47,XX,+21,47,XY,+21,Down Syndrome, Partial Trisomy 21,Down's Syndrome,Partial Trisomy 21 Down Syndrome,Trisomy 21, Meiotic Nondisjunction,Trisomy 21, Mitotic Nondisjunction,Trisomy G,Downs Syndrome,Syndrome, Down,Syndrome, Down's
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D015160	Hydrops Fetalis	Abnormal accumulation of serous fluid in two or more fetal compartments, such as SKIN; PLEURA; PERICARDIUM; PLACENTA; PERITONEUM; AMNIOTIC FLUID. General fetal EDEMA may be of non-immunologic origin, or of immunologic origin as in the case of ERYTHROBLASTOSIS FETALIS.	Edema, Fetal,Fetal Edema,Fetal Hydrops,Familial Non-Immune Hydrops Fetalis,Hydrops Fetalis Nonimmune,Hydrops Fetalis, Idiopathic,Hydrops Fetalis, Immune,Hydrops Fetalis, Non-Immune,Hydrops Fetalis, Nonimmune,Idiopathic Hydrops Fetalis,Immune Hydrops Fetalis,Non-Immune Hydrops Fetalis,Familial Non Immune Hydrops Fetalis,Fetali, Idiopathic Hydrops,Fetalis Nonimmune, Hydrops,Fetalis Nonimmunes, Hydrops,Fetalis, Idiopathic Hydrops,Hydrops Fetali, Idiopathic,Hydrops Fetali, Nonimmune,Hydrops Fetalis Nonimmunes,Hydrops, Fetal,Idiopathic Hydrops Fetali,Nonimmune Hydrops Fetali,Nonimmune Hydrops Fetalis,Nonimmune, Hydrops Fetalis,Nonimmunes, Hydrops Fetalis
D017809	Fatal Outcome	Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.	Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal
D038042	Myelopoiesis	Formation of MYELOID CELLS from the pluripotent HEMATOPOIETIC STEM CELLS in the BONE MARROW via MYELOID STEM CELLS. Myelopoiesis generally refers to the production of leukocytes in blood, such as MONOCYTES and GRANULOCYTES. This process also produces precursor cells for MACROPHAGE and DENDRITIC CELLS found in the lymphoid tissue.