BIOMAT Database Logo BIOMAT Database Logo

[Familial erythrophagocytic lymphohistiocytosis with neonatal presentation]. 1982

A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá

A neonatal case of familial erythrophagocytic lymphohistiocytosis is presented. Evolution was rapidly fatal under a course in which fever, hepatosplenomegaly, pancytopenia, severe coagulation alteration and jaundice were especially significant. Pathology findings after nine days of life, were all conclusive. Diagnostic difficulties of this uncommon entity in its' congenital form only once previously reported, are emphasized.

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
D007567 Jaundice, Neonatal Yellow discoloration of the SKIN; MUCOUS MEMBRANE; and SCLERA in the NEWBORN. It is a sign of NEONATAL HYPERBILIRUBINEMIA. Most cases are transient self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) occurring in the first week of life, but some can be a sign of pathological disorders, particularly LIVER DISEASES. Icterus Gravis Neonatorum,Neonatal Jaundice,Physiological Neonatal Jaundice,Severe Jaundice in Neonate,Severe Jaundice in Newborn,Jaundice, Physiological Neonatal,Neonatal Jaundice, Physiological
D008198 Lymph Nodes They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system. Lymph Node,Node, Lymph,Nodes, Lymph
D008206 Lymphatic Diseases Diseases of LYMPH; LYMPH NODES; or LYMPHATIC VESSELS. Lymphatism,Status Lymphaticus,Disease, Lymphatic,Diseases, Lymphatic,Lymphatic Disease
D001853 Bone Marrow The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. Marrow,Red Marrow,Yellow Marrow,Marrow, Bone,Marrow, Red,Marrow, Yellow
D004912 Erythrocytes Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN. Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D005260 Female Females
D006470 Hemorrhage Bleeding or escape of blood from a vessel. Bleeding,Hemorrhages
D006529 Hepatomegaly Enlargement of the liver. Enlarged Liver,Liver, Enlarged
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

Related Publications

A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
[Familial erythrophagocytic lymphohistiocytosis].
January 1992, Pediatriia,
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
Familial erythrophagocytic lymphohistiocytosis.
February 1991, Seminars in oncology,
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
Familial erythrophagocytic lymphohistiocytosis.
June 1977, The Journal of the Medical Society of New Jersey,
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
FAMILIAL ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS.
November 1963, Pediatrics,
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
Familial erythrophagocytic lymphohistiocytosis.
October 1984, Tijdschrift voor kindergeneeskunde,
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
Immunodeficiency in familial erythrophagocytic lymphohistiocytosis.
October 1978, Lancet (London, England),
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
[Familial erythrophagocytic lymphohistiocytosis (author's transl)].
January 1977, Anales espanoles de pediatria,
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
Immunodeficiency in familial erythrophagocytic lymphohistiocytosis.
March 1978, Lancet (London, England),
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
Familial erythrophagocytic lymphohistiocytosis in infancy.
May 1981, European journal of pediatrics,
A Arregui Sierra, and A Arbelo López de Letona, and J Quero Jiménez, and J Pérez Rodríguez, and J Alba Losada, and A Acebedo Barberá
Familial erythrophagocytic lymphohistiocytosis. A neuropathologic study.
January 1985, Acta neuropathologica,
Copied contents to your clipboard!