This study indicates that Th activation may be a red cell developmental marker present in congenital hypoplastic anemias and also expressed on newborn red cells. [ncbi.nlm.nih.gov]

• Pallor

  The typical patient presents in infancy (median 1 month of age) with pallor. In as many as 30% of the patients there is an inconsistent pattern of congenital anomalies of the heart, integument, musculoskeletal system, and kidneys. [ncbi.nlm.nih.gov]

  Chapter 442 Congenital Hypoplastic Anemia (Diamond-Blackfan Anemia) Congenital hypoplastic anemia (Diamond-Blackfan anemia, DBA) is a rare condition that usually becomes symptomatic in early infancy, often presenting with pallor in the neonatal period [clinicalgate.com]

  All had severe pallor at presentation, with mild hepato-megaly and just palpable spleen in one child. Thumb anomaly was present in one of them. [indianpediatrics.net]

  There is usually a waxy pallor, and hemorrhages occur in the mucous membranes, skin, and other organs. [britannica.com]

  Although pallor of the skin is a sign of anemia, it is not the most reliable sign; many other factors can affect complexion and skin color. [medical-dictionary.thefreedictionary.com]

• Pediatric Disease

  Succinct, targeted coverage of normal childhood growth and development, as well as the diagnosis, management, and prevention of common pediatric diseases and disorders, make this an ideal medical reference book for students, pediatric residents, nurse [books.google.com]

See Workup for more detail. Management Severe or very severe aplastic anemia is a hematologic emergency, and care should be instituted promptly. Clinicians must stress the need for patient compliance with therapy. [emedicine.medscape.com]

• Thrombocytosis

  The disease is characterized by a moderate to severe macrocytic anemia, occasional neutropenia or thrombocytosis, a normocellular bone marrow with erythroid hypoplasia, and an increased risk of developing leukemia. [journals.lww.com]

• Howell-Jolly Bodies

  Peripheral blood smears revealed Howell-Jolly bodies and postmortem examination revealed the absence of the spleen, splenic vein and splenic artery. [ncbi.nlm.nih.gov]

The content of Advances in Congenital Hypoplastic Anemia Research and Treatment / 2012 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. [books.google.com]

The second aim of the treatment is to normalize blood indicators to block the symptoms of the disease. Main directions in aplastic anemia treatment are: Immunosuppressive therapy. [bookinghealth.com]

In this paper we will show the results of such treatment, the conclusions we draw concerning the presence or failure of [jamanetwork.com]

Early differentiation of TEC and CHA appears feasible, allowing prompt provision of a favorable prognosis and the avoidance of unnecessary corticosteroid therapy in TEC. [ncbi.nlm.nih.gov]

[…] of this disorder depends upon the severity of the syndrome Please find comprehensive information on Aase Syndrome regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, [dovemed.com]

[…] association with certain viral infections (hepatitis - A, B, C; EBV, HIV, parvovirus), drugs (chloramphenicol) and toxins (benzene) Possible association with paroxysmal nocturnal hemoglobinuria May have clonal anomalies (e.g. trisomy 8) Treatment and prognosis [pathologyoutlines.com]

Prognosis Aplastic anaemia has a varied clinical course; some patients have mild symptoms that necessitate little or no therapy, whereas others present with life-threatening pancytopenia representing a medical emergency [ 7 ]. [patient.info]

For those responding to steroid therapy the prognosis is good. [medical-dictionary.thefreedictionary.com]

A progressive anemia of unknown etiology encountered in the first year of life, characterized by deficiency of red cell precursors in an otherwise normally cellular bone marrow ; it is unresponsive to hematinics and often requires multiple blood transfusions [ndif.org]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

Etiology IDA is caused by inadequate iron intake, malabsorption of iron, blood loss, pregnancy and lactation, intravascular hemolysis, or a combination of these factors. [medical-dictionary.thefreedictionary.com]

[…] million; if strictly defined, probably 2 cases/million Either constitutional / congenital or acquired (substantially more common) Most patients are adults or elderly in acquired cases; the congenital ones generally manifest during childhood Multifactorial etiology [pathologyoutlines.com]

Content ranges from the basic, to the translational, and from the epidemiology of acquired aplastic anemia and telomere biology, to the management, treatment, and supportive care of pediatric, adult, and geriatric patients. [elsevier.com]

Epidemiology [ 1, 2 ] The incidence is 2-3 per million per year in Europe, but higher in East Asia. There is a biphasic distribution, with peaks at 10-25 years and over 60 years. [patient.info]

The Diamond Blackfan Anemia Registry: tool for investigating the epidemiology and biology of Diamond Blackfan anemia. J Pediatr Hematol Oncol 2001; 23: 377-382. 4. Dianzani I, Garelli E, Ramenghi U. Diamond-Blackfan Anemia: an overview. [indianpediatrics.net]

Calado, and Phillip Scheinberg, Blood Journal, October, 2006  www.hematologica.org - Epidemiology of acquired aplastic anemia, Neal S.Young & DavidW. [slideshare.net]

Epidemiology United States statistics No accurate prospective data are available regarding the incidence of aplastic anemia in the United States. [emedicine.medscape.com]

Although its congenital nature is firmly established, the pathophysiology is still unknown, and it is likely that there are multiple biochemical or cellular defects producing the same clinical syndrome. [ncbi.nlm.nih.gov]

GhaiTextbook of Paediatrics, page 312, 313  www.bloodjournal.org - Current concepts in pathophysiology & treatment of aplastic anemia, Neal S.Young, RodrigoT. [slideshare.net]

Renal Disease hypoplastic bone marrow Multiple factors interacting with each other Decreased EPO production Presence of erythropoiesis inhibitors Decreased red cell survival Blood loss Iron deficiency Folate deficiency are the pathophysiology of anemia [quizlet.com]

[…] million Either constitutional / congenital or acquired (substantially more common) Most patients are adults or elderly in acquired cases; the congenital ones generally manifest during childhood Multifactorial etiology with numerous possible overlapping pathophysiologic [pathologyoutlines.com]

The pathophysiology is believed to be idiopathic [ 2 ] or immune-mediated phenomenon with active destruction of haematopoietic stem cells [ 3 ]. [virologyj.biomedcentral.com]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

Measures to prevent infection are explained, and the importance of adequate rest is emphasized. [medical-dictionary.thefreedictionary.com]

If platelet count is low (less than 20,000/mm3), preventing hemorrhages is vital and includes such measures as avoiding IM injections, using an electric razor and a soft toothbrush, and using humidifying oxygen to prevent drying of the mucous membranes [innvista.com]