Acquired pure red cell aplasia
Acquired pure red cell aplasia
Classification & external resources
| ICD-10 |
D60. |
| ICD-9 |
284.8 |
| DiseasesDB |
29063 |
| eMedicine |
med/1967 |
| MeSH |
D012010 |
Acquired pure red cell aplasia (or PRCA) refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells.
Causes
Pure red cell aplasia is regarded as an autoimmune disease. It may also be a manifestation of thymoma. It may also be as a result of viral infections such as HIV, herpes, parvovirus B19 (Fifth disease), or hepatitis. Association of pure red cell aplasia with T large granular lymphocyte leukemia is also well recognized, especially in China.[1] Many cases of PRCA are considered idiopathic in that there is no discernable cause detected.
It can be associated with the administration of erythropoietin.
Treatment
PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as ciclosporin. It has also been shown to respond to treatments with Rituxan.
See also
References
- ^ Association of pure red cell aplasia with T large granular lymphocyte leukaemia -- Kwong and Wong 51 (9): 672 -- Journal of Clinical Pathology. Retrieved on 2007-11-07.
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Pathology: hematology (primarily C81-C96/200-208, D45-D47, D50-D77/280-289) |
| WBCs |
hematological malignancy (lymphoma, leukemia, multiple myeloma), myeloproliferative disease, myelodysplastic syndrome
-cytosis (Agranulocytosis, Leukocytosis, Lymphocytosis, Monocytosis) • -penia (Lymphopenia, Neutropenia) |
RBCs/anemia/
hemoglobinopathy |
nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia)
hereditary hemolytic anemia: G6PD Deficiency, Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis
acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH
aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis |
| Coagulation/platelets |
coagulopathy: DIC • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease
Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP
primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Antiphospholipid syndrome - Factor V Leiden
other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome |
| Histiocytosis |
WHO-I Langerhans cell histiocytosis - non-Langerhans-cell histiocytosis/WHO-II (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) - malignant histiocytic disorders/WHO-III (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) |
| Other |
Asplenia/hyposplenism - Methemoglobinemia |
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