先天性非球状赤血球性溶血性貧血
WordNet
- present at birth but not necessarily hereditary; acquired during fetal development (同)inborn, innate
- relating to or involving or causing hemolysis; "hemolytic anemia" (同)haemolytic
- a deficiency of red blood cells (同)anaemia
- a lack of vitality (同)anaemia
- genus of terrestrial or lithophytic ferns having pinnatifid fronds; chiefly of tropical America (同)genus Anemia
PrepTutorEJDIC
- (病気・身体的欠陥など)生まれつきの,先天的な
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/04/19 09:44:05」(JST)
[Wiki en表示]
| Congenital hemolytic anemia |
| Classification and external resources |
| ICD-10 |
D55-D58 |
| ICD-9 |
282 |
| MeSH |
D000745 |
Congenital hemolytic anemia (or hereditary hemolytic anemia) refers to hemolytic anemia which is primarily due to congenital disorders.[1]
Types
Basically classified by causative mechanism, types of congenital hemolytic anemia include:
- Genetic conditions of RBC Membrane
- Hereditary spherocytosis
- Hereditary elliptocytosis
- Genetic conditions of RBC metabolism (enzyme defects). This group is sometimes called congenital nonspherocytic (hemolytic) anemia, which is a term for a congenital hemolytic anemia without spherocytosis, and usually excluding hemoglobin abdormalities as well, but rather encompassing defects of glycolysis in the erythrocyte.[2]
- Glucose-6-phosphate dehydrogenase deficiency (G6PD or favism)
- Pyruvate kinase deficiency
- Aldolase A deficiency
- Hemoglobinopathies[3]/genetic conditions of hemoglobin
- Sickle cell anemia
- Congenital dyserythropoietic anemia
- Thalassemia
See also
- Hematopoietic ulcer
- List of circulatory system conditions
References
- ^ Rahman SA, Jamal CY (June 2002). "Congenital hemolytic anemia in Bangladesh: types and clinical manifestations". Indian Pediatr 39 (6): 574–7. PMID 12084953.
- ^ medconditions.net > Hemolytic Congenital, Nonspherocytic Anemia Definition Retrieved April 15, 2011
- ^ Shah A (November 2004). "Hemoglobinopathies and other congenital hemolytic anemia". Indian J Med Sci 58 (11): 490–3. PMID 15567909.
|
Pathology: hematology, hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287)
|
|
Red
blood cells |
| ↑ |
|
|
| ↓ |
| Anemia |
| Nutritional |
- Micro-: Iron deficiency anemia
- Macro-: Megaloblastic anemia
|
|
Hemolytic
(mostly Normo-) |
| Hereditary |
- enzymopathy: G6PD
- glycolysis
- hemoglobinopathy: Thalassemia
- Sickle-cell disease/trait
- HPFH
- membrane: Hereditary spherocytosis
- Minkowski-Chauffard syndrome
- Hereditary elliptocytosis
- Southeast Asian ovalocytosis
- Hereditary stomatocytosis
|
|
| Acquired |
- Drug-induced autoimmune
- Drug-induced nonautoimmune
- Hemolytic disease of the newborn
|
|
|
Aplastic
(mostly Normo-) |
- Hereditary: Fanconi anemia
- Diamond–Blackfan anemia
- Acquired: PRCA
- Sideroblastic anemia
- Myelophthisic
|
|
| Blood tests |
- MCV
- Normocytic
- Microcytic
- Macrocytic
- MCHC
|
|
|
| Other |
- Methemoglobinemia
- Sulfhemoglobinemia
- Reticulocytopenia
|
|
|
|
Coagulation/
coagulopathy |
| ↑ |
Hyper-
coagulability |
- primary: Antithrombin III deficiency
- Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden
- Prothrombin G20210A
- Sticky platelet syndrome
- acquired:Thrombocytosis
- DIC
- Congenital afibrinogenemia
- Purpura fulminans
- autoimmune
|
|
|
| ↓ |
Hypo-
coagulability |
| Thrombocytopenia |
- Thrombocytopenic purpura: ITP
- TM
- Heparin-induced thrombocytopenia
- May-Hegglin anomaly
|
|
| Platelet function |
- adhesion
- aggregation
- Glanzmann's thrombasthenia
- platelet storage pool deficiency
- Hermansky–Pudlak syndrome
- Gray platelet syndrome
|
|
| Clotting factor |
- Hemophilia
- von Willebrand disease
- Hypoprothrombinemia/II
- XIII
- Dysfibrinogenemia
|
|
|
|
|
|
|
cell/phys (coag, heme, immu, gran), csfs
|
rbmg/mogr/tumr/hist, sysi/epon, btst
|
drug (B1/2/3+5+6), btst, trns
|
|
|
|
UpToDate Contents
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English Journal
- Diagnosis of Pyruvate Kinase Deficiency.
- Gallagher PG1, Glader B2,3.
- Pediatric blood & cancer.Pediatr Blood Cancer.2016 May;63(5):771-2. doi: 10.1002/pbc.25922. Epub 2016 Feb 2.
- PMID 26836632
- Two Novel Missense Mutations and a 5bp Deletion in the Erythroid-Specific Promoter of the PKLR Gene in Two Unrelated Patients With Pyruvate Kinase Deficient Transfusion-Dependent Chronic Nonspherocytic Hemolytic Anemia.
- Kager L1,2, Minkov M1,3, Zeitlhofer P4, Fahrner B1, Ratzinger F5, Boztug K1,6, Dossenbach-Glaninger A7, Haas OA1,2,4.
- Pediatric blood & cancer.Pediatr Blood Cancer.2016 May;63(5):914-6. doi: 10.1002/pbc.25878. Epub 2016 Jan 5.
- We report two children with severe chronic hemolytic anemia, the cause of which was difficult to establish because of transfusion dependency. Reduced erythrocyte pyruvate kinase activity in their asymptomatic parents provided the diagnostic clues for mutation screening of the PKLR gene and revealed
- PMID 26728349
- PMID 27031109
Japanese Journal
- A Case of Congenital Nonspherocytic Hemolytic Anemia Associated with Glucosephosphate Isomerase (GPI) Deficiency-GPI ′Kinki′
- Three Cases in Two Families with Congenital Nonspherocytic Hemolytic Anemia due to Defective Glucosephosphate Isomerase--GPI Matsumoto
- Congenital nonspherocytic hemolytic anemia due to glucose-6-phosphate dehydrogenase deficiency in a japanese child Clinical and metabolic studies
Related Links
- Information about congenital nonspherocytic hemolytic anemia in the Columbia Encyclopedia, Computer Desktop Encyclopedia, computing dictionary. Printer Friendly Dictionary, Encyclopedia and Thesaurus - The Free Dictionary ...
- Definition of congenital nonspherocytic hemolytic anemia in the Medical Dictionary. congenital nonspherocytic hemolytic anemia explanation. Information about congenital nonspherocytic hemolytic anemia in Free online English ...
★リンクテーブル★
[★]
- 英
- congenital nonspherocytic hemolytic anemia
- 関
- 先天性非球状赤血球性溶血性貧血
[★]
- 英
- congenital nonspherocytic hemolytic anemia
[★]
- 関
- congenital、congenitally
[★]
- 関
- haemolysis、haemolytic、hemolysis、hemolyze
[★]