汎血球減少
WordNet
- an abnormal deficiency in all blood cells (red blood cells and white blood cells and platelets); usually associated with bone marrow tumor or with aplastic anemia
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/08/24 22:15:30」(JST)
[Wiki en表示]
| Pancytopenia |
| Classification and external resources |
| ICD-10 |
D61.9 |
| ICD-9 |
284.1 |
| DiseasesDB |
24135 |
| MeSH |
D010198 |
Pancytopenia is a medical condition in which there is a reduction in the number of red and white blood cells, as well as platelets.
If only two parameters from the full blood count are low, the term bicytopenia can be used. The diagnostic approach is the same as for pancytopenia.
Contents
- 1 Definition
- 2 Causes
- 3 Diagnosis
- 4 References
- 5 External links
Definition
- anemia: hemoglobin < 13.5 g/dL (male) or 12 g/dL (female).
- leukopenia: total white cell count < 4.0 x 109/L. Decrease in all types of white blood cells (revealed by doing a differential count).
- thrombocytopenia: platelet count < 150×109/L.
The disease is marked by an inappropriate and ineffective T cell activation that leads to an increased hemophagocytic activity. The T cell activated macrophages engulf erythrocytes, leukocytes, platelets, as well as their progenitor cells. Such finding is common in the syndrome, which is also referred to as hemophagocytic lymphohistiocytosis (HLH). Along with pancytopenia, HLH is characterized by fever, splenomegaly, and hemophagocytosis in bone marrow, liver, or lymph nodes.
Iatrogenic causes of pancytopenia include chemotherapy for malignancies if the drug or drugs used cause bone marrow suppression. Rarely, drugs (antibiotics, blood pressure medication, heart medication) can cause pancytopenia. The antibiotics Linezolid and Chloramphenicol can cause pancytopenia in some individuals.
Rarely, pancytopenia may have other causes, such as mononucleosis, or other viral diseases. Increasingly, HIV is itself a cause for pancytopenia.
Causes
- Familial hemophagocytic syndrome
- Aplastic anemia
- Gaucher's disease
- metastatic carcinoma of bone
- multiple myeloma
- overwhelming infections
- lymphoma
- myelofibrosos
- Dyskeratosis congenita
- Myelodysplastic syndrome
- Leukemia
- Leishmaniasis
- Severe Folate or vitamin B12 deficiency
- Systemic lupus erythematosus
- Paroxysmal nocturnal hemoglobinuria (urine test)
- viral infections (HIV most common).
- Alimentary toxic aleukia
- Copper deficiency
- Pernicious anemia
- Medication
- Hypersplenism
- Osteopetrosis
- Organic acidurias (Propionic Acidemia, Methylmalonic Aciduria, Isovaleric Aciduria)
- Low dose arsenic poisoning
- Sako disease (Myelodysplastic-cytosis)
- Chronic radiation sickness[1]
- LIG4 syndrome
Diagnosis
Pancytopenia usually requires a bone marrow biopsy in order to distinguish among different causes.
References
- ^ Kossenko, M.M; Akleyev. A.A., Degteva. M.O., Kozheurov. V.P., Degtyaryova. R.C. (August 1994). "Analysis of Chronic Radiation Sickness Cases in the Population of the Souther Urals (AD-A286 238)". DTIC. p. 5. Retrieved 1 August 2013. "Complete blood counts, when taken, revealed pancytopenia."
|
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
|
|
|
|
External links
|
Wikimedia Commons has media related to Hemophagocytic Infections and Syndromes. |
- EID Journal (Volume 6, Number 6), CDC, December 2000.
- Aetiopancyto – Université Angers
UpToDate Contents
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English Journal
- A case of recurrent pancytopenia in a patient with acute promyelocytic leukemia on maintenance chemotherapy and concomitant methyltetrahydrofolate reductase and thiopurine S-methyltransferase mutation - review of literature.
- Keung YK1, Keung LW2, Hong-Lung Hu E2.
- Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners.J Oncol Pharm Pract.2016 Jun;22(3):548-51. doi: 10.1177/1078155215577235. Epub 2015 Mar 18.
- Pharmacogenetics is a study of how genetic variation of an individual affects the drug response. We report a case of recurrent pancytopenia resulting from maintenance chemotherapy in a patient with acute promyelocytic leukemia and two pharmacogenetic mutations, namely, methylene tetrahydrofolate red
- PMID 25791511
- Bone Marrow Oxalosis in a Patient With Pancytopenia Following Bilateral Nephrectomy.
- Kim MJ1, Park PW1, Seo YH1, Kim KH1, Seo JY1, Jeong JH1, Lee HT1, An J2, Ahn JY3.
- Annals of laboratory medicine.Ann Lab Med.2016 May;36(3):266-7. doi: 10.3343/alm.2016.36.3.266.
- PMID 26915618
- Pure Red Cell Aplasia in Major ABO-Mismatched Allogeneic Hematopoietic Stem Cell Transplantation Is Associated with Severe Pancytopenia.
- Aung FM1, Lichtiger B1, Rondon G2, Yin CC3, Alousi A2, Ahmed S2, Andersson BS2, Bashir Q2, Ciurea SO2, Hosing C2, Jones R2, Kebriaei P2, Khouri I2, Nieto Y2, Oran B2, Parmar S2, Qazilbash M2, Shah N2, Shpall EJ2, Champlin RE2, Popat U4.
- Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.Biol Blood Marrow Transplant.2016 May;22(5):961-5. doi: 10.1016/j.bbmt.2016.02.008. Epub 2016 Feb 26.
- In major ABO-mismatched allogeneic hematopoietic stem cell transplantation (HSCT) persistence of antidonor isohemagglutinins leads to pure red cell aplasia (PRCA). To investigate severe pancytopenia noted in a previous study of PRCA, we analyzed all major ABO-mismatched HSCT between January 2003 and
- PMID 26921820
Japanese Journal
- 妊娠初期に血球貪食症候群を発症したが健常児を得た1例
- 神奈川産科婦人科学会誌 = Kanagawa journal of obstetrics and gynecology : official journal, Kanagawa Society of Obstetrics and Gynecology 53(2), 163-166, 2017-02
- NAID 40021115774
- Hassab手術（脾臓摘出術および胃上部血行郭清術）が奏効した進行性の汎血球減少症と食道胃静脈瘤を有する非アルコール性脂肪肝炎（NASH）の1例
Related Links
- UpToDate, electronic clinical resource tool for physicians and patients that provides information on Adult Primary Care and Internal Medicine, Allergy and Immunology, Cardiovascular Medicine, Emergency Medicine, Endocrinology ...
- What is pancytopenia, what are the symptoms, what causes it, and how is it treated? Let's take a look at those questions. Definition: Pancytopenia Pancytopenia is defined as abnormally low levels of all blood cells ...
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