Osteomyelofibrosis
| Myelofibrosis | |
|---|---|
| A classification and outside reference information | |
| Clinical department ・ Academic field | Blood internal medicine, tumor internal medicine |
| ICD-10 | C94.4, D47.4 |
| ICD-9-CM | 289.83 |
| ICD-O | 9932, M9961/3 |
| OMIM | 254450 |
| DiseasesDB | 8616 |
| MedlinePlus | 000531 |
| Patient UK | Osteomyelofibrosis |
| MeSH | D055728 |
Osteomyelofibrosis (こつずいせんいしょう, fame Myelofibrosis) is blood disorder that marrow becomes fibrotic, and normal hematopoiesis is affected [1].
Is an intractable disease appointed by specific disease of Ministry of Health, Labour and Welfare (but is not a disease for the specific disease treatment study business with the furtherance of medical expenses); [2].
Table of contents
Classification
With a primary thing called idiopathic osteomyelofibrosis or the primary osteomyelofibrosis (fame Idiopathic myelofibrosis) and the secondary thing based on the underlying disease of various blood tumors and collagenosis, bone diseases, the bone metastasis of cancer, many other kinds [1] [3]. The gene variation of the hematopoietic stem cells level produces the idiopathic osteomyelofibrosis, and it is basic that blood corpuscles (mainly macronuclei ball) which are a neoplastic clone multiply, and the genuine plethora that is the neoplastic clone addability disease of the hematopoietic stem cells level and essential thrombocythemia are near essentially in the same way [4]. Therefore included in the disease myeloproliferative with genuine plethora and essential thrombocythemia, chronic myelogenous leukemia [5]. Because the secondary thing may have various variation by an underlying disease, I explain idiopathy osteomyelofibrosis in this report mainly as follows.
Summary
I am characterized by marrow fibrosis, splenomegalia, white erythroblastosis (fame leukoerythroblastosis), marrow outside hematopoiesis [6] and am blood disorder to bring high anemia and thrombopenia when there is it, and slight anemia progresses early [1].
Myeloid fibrosis depends on a fibroblastic increase, but a multiplying fibroblast is a normal cell, and it is thought with the increase that the increase is many clonal expansion, and is reactive [6]. It is thought that the cause of the normal fibroblastic abnormal increase is stimulation with cytokine which the blood corpuscles such as abnormal macronuclei balls produce, and blood corpuscles such as abnormal macronuclei balls are tumor clones of the hematopoietic stem cells level, and megakaryocytic increase is seen with myeloid fibrosis [6].
Because marrow becomes fibrotic normal hematopoiesis is obstructed, and hematopoiesis comes to be carried out other than marrow (hematopoiesis out of the marrow, extramedullary hematopoiesis) particularly is made blood in liver, spleen, liver, the spleen swells [7]. Spleen calls a swollen state splenomegalia, and, as for the osteomyelofibrosis patient, most cause splenomegalia [4]. I often cause huge splenomegalia, and there is 巨脾 accounting for abdominal most among them [8].
As a result of hematopoiesis out of the marrow, erythroblast, a myeloblast come to be recognized in peripheral blood. The phenomenon to show erythroblast, the myeloblast which are not seen is named white erythroblastosis with the peripheral blood of the healthy person [4].
Etiology
Myeloid fibrosis depends on a fibroblastic increase, but a multiplying fibroblast is a normal cell, and the increase is thought about with a reactive increase. It is thought that the cause of the normal fibroblastic abnormal increase is stimulation with cytokine which the blood corpuscles such as abnormal macronuclei balls produce [6], but the cause that the blood corpuscles such as abnormal macronuclei balls occur is unidentified. The point mutation of the tyrosine kinase JAK2 gene is discovered in half of the idiopathic osteomyelofibrosis and is called JAK2V617F variation fused gene, and causation with the onset has it pointed out.
Symptom
It is not rare to be a diagnosis at all times symptom, and is often found from the disorder of splenomegalia and the blood test [6]; [7]. When a lot of anemia symptoms such as a heartbeat, shortness of breath, the general malaise move for the early stage of symptom, an anemia symptom becomes strong, and abdominal acute pains by abdominal distension feeling of, 脾梗塞 by inappetence, the splenomegalia may get up again [6]; [9].
Inspection
As for the inspection, abdominal inspection such as CT and MRI, the echo check to see examination of marrow, blood test, 脾肝腫 is performed.
It is bone marrow aspiration by an examination of marrow and is often dry tap, and there is see degree of fibrosis again, collecting it, and marrow biopsy is necessary. Because fibrosis does not advance uniformly, and there is an irregularity, I doubt it, and what marrow performs a biopsy on in a different part may be necessary for a patient [6].
Histopathological views
The feature is that anemia, white erythroblastosis and teardrop-shaped red blood cells are seen with the peripheral blood. Anemia is normocytic normochromic anemia. Anemia becomes strong with progress. The white blood cell often does slight increase, but may decrease. The platelets often increase early, but often decrease when it progresses. A disorder is seen in the platelet functionally morphologically. There are many a thing and platelet function drops that a huge platelet is seen in [4]; [6].
It is a hyperplasia, and polymorph and macronuclei balls increase with the marrow extremely early, but it is rare that a disease is discovered extremely early. It is in a condition that fibrosis advances with the marrow, and a hematopoietic ingredient decreased, and a symptom appears and has a medical examination and is usually discovered. It is seen rise an atypical macronuclei ball at the same time, and to make an agglomeration, and admit by the increase of the again bone beam [4]; [6].
Epidemiology
According to the survey by survey by Ministry of Health and Welfare study group about the idiopathic hematopoietic disorder, the case from the estimated newcomer in Japan is 60-70 a year, and, as for the onset age median, 65 years old, the sex ratio are 1.64:1 [1]. However, as for the annual onset rate, there are many things considered to be one of 100,000 degree in 成書 [6] and the American study [9]. The difference is thought to be a thing by the closeness of the diagnostic criteria application.
Diagnostic criteria
1.As a clinical evidence
- I accept anemia and hepatosplenomegaly to gradually progress.
- By the way, I accept bleeding tendency, abdominal feeling of distension, fever, general malaise, weight loss.
- I cause portal vein pressure sthenia, abdominal dropsy when it progresses.
2.I accept the following laboratory findings.
- I see white erythroblastosis, the malformed red blood cell, blast cells such as teardrop red blood cells, a huge platelet, a megakaryocytic appearance with peripheral blood.
- A rise of serum LDH which does not accept a cause elsewhere.
- I accept significant hepatosplenomegaly by an examination for image.
- In marrow scintigraphy, I recognize uptake increase to 肝脾.
- I cannot gather a bone cerebrospinal fluid by bone marrow aspiration (dry tap).
3.I recognize atypical macronuclei ball increase and myeloid fibrosis, the increase of the bone beam by marrow biopsy.
4.I exclude secondary osteomyelofibrosis.
On the occasion of a diagnosis
I doubt primary osteomyelofibrosis by one or two,
I confirm myeloid fibrosis by 3.
It shall be more certain by a diagnosis excluding secondary osteomyelofibrosis by 4.
By (public welfare labor science study intractable disease conquest study business 2004) [4].
Treatment
Basic treatment is similar hematopoietic stem cells transplant such as the bone marrow transplantation, but the average age of disease patients is high, and there are few patients who can be adapted to of similar hematopoietic stem cells transplant [3]; [4]. In addition, I do not fit a patient with a few poor-prognosis genes even if it is a young patient (reference with the next gnarl about the poor-prognosis gene). Therefore the treatment is made up mainly of the management of complications [3].
With the report that a steroid and the chemotherapy (including Hyde rare, etoposide) are used for measures of improvement and the splenomegalia of the blood views including anemia measures, but a protein assimilation hormone (タナゾール), melphalan and thalidomide are effective [10].
If anemia and thrombopenia are high, the blood transfusion is provided, too.
If a symptom by 巨脾 and 脾梗塞 is strong, I splenectomize it, and there is the radiation therapy to spleen, too [7]. But I splenectomize it, and the radiation therapy to spleen may exacerbate anemia.
The innovative drug development that targeted JAK2V617F variation fused gene is studied, and ルキソリチニブ which is the tyrosine kinase repressor which assumes JAK1/JAK2 a target is authorized as a therapeutic drug for the primary osteomyelofibrosis in 2014.
Convalescence
It is ten years from five years [7] after the diagnosis of the disease patient for the mean length of life [1].
As for the main cause of death, 33% of infectious diseases, white blood are 19%, heart failure 12%, order of 11% of cerebrovascular disorder [4].
It is reported to the survival rate with 31% in the case to have more than 84%, two to anemia less than (1)Hb 10 g/dL, the continuation of the symptoms such as (2) fever, sweat, the weight loss, (3) peripheral blood for a poor-prognosis gene for ten years of the case that only lower than have 挙 げらるが, one these poor-prognosis factors that it is the appearance of more than 1% of blast cells, (4) man [4]. A transformation rate to leukemia is less than 30%, but has a poor prognosis when I transform.
The source, footnote
- ^ a b c d e Osteomyelofibrosis intractable disease information center intractable disease medicine study foundation Foundation, Ministry of Health, Labour and Welfare
- ^ Intractable disease information center HP top intractable disease information center intractable disease medicine study foundation Foundation, Ministry of Health, Labour and Welfare
- ^ a b c Shigetaka Asano, Taku Uchiyama, the Yasuo Ikeda supervision, "the three-wheeled blood disease studies third edition," it is sentence Amitabha hall, 2006, P949, ISBN 4-8306-1419-6
- ^ a b c d e f g h i Hospital blood internal medicine, blood disorder commentary, nuclear power generation-related osteomyelofibrosis attached to the Osaka City University medical department
- ^ Commentary, chronic myelogenous leukemia, chronic marrow proliferative disease of national cancer research center, various cancers
- ^ a b c d e f g h i j Shigetaka Asano, Taku Uchiyama, the Yasuo Ikeda supervision, "the three-wheeled blood disease studies third edition," it is sentence Amitabha hall, 2006, P948, ISBN 4-8306-1419-6
- ^ a b c d Commentary, osteomyelofibrosis of Juntendo Clinic attached to the Juntendo University medical department, blood internal medicine, representative-like blood disorder
- ^ Kitasato University, internal medicine diagnostic study access, splenomegalia
- ^ a b Merck manual, osteomyelofibrosis
- ^ Japan internal medicine society editing, publication, "Japanese internal medicine society magazine Vol 96 No7," it is 2007, P73
Allied item
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