今日の臨床サポート

原発性骨髄線維症

著者: 下田和哉 宮崎大学 内科学講座消化器血液学分野

監修: 木崎昌弘 埼玉医科大学総合医療センター

著者校正/監修レビュー済:2016/12/28
患者向け説明資料

概要・推奨   

疾患のポイント:
  1. 原発性骨髄線維症は、造血幹細胞レベルでの異常に起因する骨髄の線維化を来す疾患である。髄外造血による肝脾腫を伴い、末梢血では幼若な顆粒球と赤芽球が出現する白赤芽球症(leukoerythroblastosis)を認める。約半数にJAK2V617F変異、20~30%にCALR変異、数%にMPL変異を認める。
  1. 骨髄線維症は、骨髄に広範な線維化、骨硬化を来す疾患の総称であり、体重減少、全身倦怠感、瘙痒感などの全身症状、髄外造血による巨脾、腹部膨満感などの臨床症状を示す。進行すると、造血不全、白血化を来す。原発性骨髄線維症とほかの疾患に続発する2次性骨髄線維症に大別される。
  1. 貧血、末梢血への赤芽球、骨髄芽球の出現、巨大脾腫などの所見がある場合は、骨髄線維症を鑑別疾患に挙げる。
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  1. 骨髄生検を行い骨髄の広範な線維化を伴う巨核球の増殖と異形成、骨梁の増加を認めて確定する。骨髄の線維化は、原発性骨髄線維症のみならず、、などに伴って生じることもあるので、2次性の骨髄線維症の除外が必須となる。造血細胞がクローナルな(腫瘍性の)細胞増殖を生じていることを、JAK2V617F変異などにより確認する。
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  1. 髄外造血による肝脾腫を伴い、約半数の症例でJAK2V617F変異を有する。
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
下田和哉 : 講演料(ノバルティスファーマ(株),武田薬品工業(株)),研究費・助成金など(ファーマエッセンシアジャパン(株)),奨学(奨励)寄付など(中外製薬(株),アッヴィ合同会社,協和キリン(株),新日本先進医療研究財団,日本血液学会,ライフサイエンス振興財団)[2021年]
監修:木崎昌弘 : 講演料(ブリストル・マイヤーズスクイブ,ヤンセンファーマ,ノバルティスファーマ,セルジーン,MSD,小野薬品,武田薬品,大日本住友製薬),研究費・助成金など(武田薬品),奨学(奨励)寄付など(協和キリン,中外製薬,武田薬品,小野薬品,第一三共)[2021年]

病態・疫学・診察

疾患情報  
  1. 原発性骨髄線維症は、造血幹細胞レベルで生じた遺伝子異常により、骨髄中で巨核球をはじめとする血液細胞が増殖する難治性疾患である[1]
  1. 増加した血液細胞、特に巨核球が産生する種々のサイトカインが骨髄間質細胞に作用し、原発性骨髄線維症の特徴である全身の骨髄の線維化(<図表>)、骨硬化を生じる。
  1. 肝脾腫と肝・脾における髄外造血を伴い、末梢血では幼若な顆粒球と赤芽球が出現する白赤芽球症を認める。<図表>
  1. 骨髄の広範な線維化(<図表>)は、原発性骨髄線維症以外にも、種々の基礎疾患に続発して原発性骨髄線維症の臨床経過は均一ではなく、症例間によるばらつきが大きい。わが国の原発性骨髄線維症の5年生存率は41%、生存期間中央値は4.0年である[3][4]
問診・診察のポイント  
  1. 体重減少、発熱、 盗汗 など、全身症状の有無を確認する。

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文献 

著者: T Okamura, N Kinukawa, Y Niho, H Mizoguchi
雑誌名: Int J Hematol. 2001 Feb;73(2):194-8.
Abstract/Text We retrospectively analyzed 336 patients with primary chronic myelofibrosis from 203 medical institutes in Japan. Notwithstanding their heterogeneous treatments, the median survival in 298 patients that could be evaluated was 10.0 years. Average age at onset was 60.7 years. Men were affected 1.4 times more frequently than women. The factors associated with shorter survival included anemia, leukocytosis/leukocytopenia, thrombocytopenia, and increased blasts in the peripheral blood, and sex (male), age (>60), and the presence of symptoms. A new scoring system based on the peripheral blood findings (hemoglobin [Hb] level, platelet count, and rate of blast formation) at initial diagnosis clearly correlated with survival rate. Accordingly, patients could be categorized into 3 groups by severity grading. Through stepwise multivariate survival analysis, the significant prognostic factors were identified as the severity grading based on the new scoring system (P = .0002), age (P = .0024), sex (P = .0153), and Hb (P = .0198). Chromosomal abnormalities were found in 58 of 154 patients (38%), although these did not influence survival.

PMID 11372731  Int J Hematol. 2001 Feb;73(2):194-8.
著者: Tomonori Hidaka, Kotaro Shide, Haruko Shimoda, Takurou Kameda, Keiko Toyama, Keiko Katayose, Youko Kubuki, Kenji Nagata, Katsuto Takenaka, Koichi Akashi, Takashi Okamura, Yoshiyuki Niho, Hideaki Mizoguchi, Mitsuhiro Omine, Keiya Ozawa, Mine Harada, Kazuya Shimoda
雑誌名: Eur J Haematol. 2009 Oct;83(4):328-33. doi: 10.1111/j.1600-0609.2009.01298.x. Epub 2009 Jun 15.
Abstract/Text Cytogenetic abnormalities were often observed in primary myelofibrosis patients. The presence of specific cytogenetic abnormalities, such as sole abnormalities of chromosome 13q-, 20q-, or -7/7q-, is reported to have the influence on the prognosis of primary myelofibrosis. We analyzed the data from the prospective survey of Japanese primary myelofibrosis patients which was conducted from 1999 to clarify the impact of cytogenetic abnormalities on the prognosis of primary myelofibrosis. A total of 202 primary myelofibrosis patients had the cytogenetic and the prognostic data. Eighty (40%) out of 202 cases had cytogenetic abnormalities, and an association was evident for platelet counts. Although the presence of an abnormal karyotype did not affect the prognosis, primary myelofibrosis patients with cytogenetic abnormalities other than 13q- and 20q- showed an inferior prognosis compared to patients with a normal karyotype or sole 13q- or 20q- abnormalities. Patients with an unfavorable cytogenetic profile (abnormal cytogenetics other than 13q- or 20q-) also had a greater tendency to transform to leukemia than patients with a favorable cytogenetic profile (normal cytogenetics, sole abnormalities of either chromosome 13q-, or 20q-). Abnormal cytogenetics other than 13q- or 20q- in primary myelofibrosis patients has the poor prognostic effect for both survival and the risk of leukemic transformation.

PMID 19549278  Eur J Haematol. 2009 Oct;83(4):328-33. doi: 10.1111/j.1・・・
著者: Francisco Cervantes, Brigitte Dupriez, Arturo Pereira, Francesco Passamonti, John T Reilly, Enrica Morra, Alessandro M Vannucchi, Ruben A Mesa, Jean-Loup Demory, Giovanni Barosi, Elisa Rumi, Ayalew Tefferi
雑誌名: Blood. 2009 Mar 26;113(13):2895-901. doi: 10.1182/blood-2008-07-170449. Epub 2008 Nov 6.
Abstract/Text Therapeutic decision-making in primary myelofibrosis (PMF) is becoming more challenging because of the increasing use of allogeneic stem cell transplantation and new investigational drugs. To enhance this process by developing a highly discriminative prognostic system, 1054 patients consecutively diagnosed with PMF at 7 centers were studied. Overall median survival was 69 months (95% confidence interval [CI]: 61-76). Multivariate analysis of parameters obtained at disease diagnosis identified age greater than 65 years, presence of constitutional symptoms, hemoglobin level less than 10 g/dL, leukocyte count greater than 25 x 10(9)/L, and circulating blast cells 1% or greater as predictors of shortened survival. Based on the presence of 0 (low risk), 1 (intermediate risk-1), 2 (intermediate risk-2) or greater than or equal to 3 (high risk) of these variables, 4 risk groups with no overlapping in their survival curves were delineated; respective median survivals were 135, 95, 48, and 27 months (P< .001). Compared with prior prognostic models, the new risk stratification system displayed higher predictive accuracy, replicability, and discriminating power. In 409 patients with assessable metaphases, cytogenetic abnormalities were associated with shorter survival, but their independent contribution to prognosis was restricted to patients in the intermediate-risk groups. JAK2V617F did not cluster with a specific risk group or affect survival.

PMID 18988864  Blood. 2009 Mar 26;113(13):2895-901. doi: 10.1182/blood・・・
著者: Francisco Cervantes, Alberto Alvarez-Larrán, Abel Domingo, Eduardo Arellano-Rodrigo, Emili Montserrat
雑誌名: Br J Haematol. 2005 Jun;129(6):771-5. doi: 10.1111/j.1365-2141.2005.05524.x.
Abstract/Text Androgens are considered the treatment of choice for the anaemia of myelofibrosis with myeloid metaplasia (MMM). Good results have been reported in a few patients treated with danazol, a synthetic attenuated androgen. The long-term efficacy and tolerability of danazol as treatment for the anaemia of MMM was evaluated in 30 patients, who received 600 mg/d, with progressive tapering to the minimum effective dose in the responders after 6 months. Complete response (CR) was defined as transfusion cessation with normal Hb and partial response (PR) as an Hb increase >/=1.5 g/dl with transfusion-independent Hb values >10 g/dl maintained for at least 8 weeks. Median follow-up was 20.5 months (range: 3.5-58 months). Response was achieved in 11 patients (37%), including eight CRs and three PRs. Median time to response was 5 months (range: 1-9 months). Four patients stopped responding at 6-24 months, two responders discontinued treatment because of toxicity, and five maintained response at 3.5-42 months. Pretreatment variables associated with response were lack of transfusion requirement (P= 0.001) and higher Hb at treatment start (P= 0.02). Toxicity was usually moderate, leading to treatment withdrawal in only two cases. Danazol is effective and well tolerated in a substantial proportion of MMM patients with anaemia.

PMID 15953003  Br J Haematol. 2005 Jun;129(6):771-5. doi: 10.1111/j.13・・・
著者: Kazuya Shimoda, Kotaro Shide, Kenjirou Kamezaki, Takashi Okamura, Naoki Harada, Naoko Kinukawa, Kazuma Ohyashiki, Yoshiyuki Niho, Hideaki Mizoguchi, Mitsuhiro Omine, Keiya Ozawa, Mine Haradaa
雑誌名: Int J Hematol. 2007 May;85(4):338-43. doi: 10.1532/IJH97.06135.
Abstract/Text Between 1999 and 2005, 285 patients received new diagnoses of myelofibrosis with myeloid metaplasia (MMM) in Japan. Anemic symptoms were present in 162 patients, and hemoglobin (Hb) concentrations were <10 g/dL in 197 patients. Fifty-five MMM patients were treated with anabolic steroids, and their effect on anemia during MMM was evaluated in 39 patients. A "good" response was defined as an Hb increase of >or=1.5 g/dL, cessation of transfusion dependence, and an Hb concentration of >10 g/dL maintained for at least 8 weeks. A "minimum" response was defined as an Hb increase of >or=1.5 g/dL and transfusion independence for at least 8 weeks. Both good and minimum responses were considered "favorable." Favorable responses were achieved in 17 patients (44%, 8 good and 9 minimum responses). None of the pretreatment variables, such as the lack of transfusion dependence, a higher Hb concentration at the start of treatment, or the absence of cytogenetic abnormalities, were associated with a response to anabolic steroid therapy. Adverse events associated with anabolic steroid therapy were moderate and transient. Two patients required definitive withdrawal of treatment. Thus, anabolic steroids are well tolerated and effective for the treatment of anemia in a subset of MMM patients.

PMID 17483079  Int J Hematol. 2007 May;85(4):338-43. doi: 10.1532/IJH9・・・
著者: Ruben A Mesa, David P Steensma, Animesh Pardanani, Chin-Yang Li, Michelle Elliott, Scott H Kaufmann, Gregory Wiseman, Leigh A Gray, Georgene Schroeder, Terra Reeder, Jerome B Zeldis, Ayalew Tefferi
雑誌名: Blood. 2003 Apr 1;101(7):2534-41. doi: 10.1182/blood-2002-09-2928. Epub 2002 Nov 27.
Abstract/Text Single-agent thalidomide (THAL) at "conventional" doses (> 100 mg/d) has been evaluated in myelofibrosis with myeloid metaplasia (MMM) based on its antiangiogenic properties and the prominent neoangiogenesis that occurs in MMM. THAL monotherapy at such doses produces approximately a 20% response rate in anemia but is poorly tolerated (an adverse dropout rate of > 50% in 3 months). To improve efficacy and tolerability, we prospectively treated 21 symptomatic patients (hemoglobin level < 10 g/dL or symptomatic splenomegaly) with MMM with low-dose THAL (50 mg/d) along with a 3-month oral prednisone (PRED) taper (beginning at 0.5 mg/kg/d). THAL-PRED was well tolerated in all enrolled patients, with 20 patients (95%) able to complete 3 months of treatment. An objective clinical response was demonstrated in 13 (62%) patients, all improvements in anemia. Among 10 patients who were dependent on erythrocyte transfusions, 7 (70%) improved and 4 (40%) became transfusion independent. Among 8 patients with thrombocytopenia (platelet count < 100 x 10(9)/L), 6 (75%) experienced a 50% or higher increase in their platelet count. In 4 of 21 patients (19%), spleen size decreased by more than 50%. Responses observed were mostly durable after discontinuation of the PRED. The dose of THAL in this study (50 mg/d) was better tolerated than the higher doses used in previous studies. Adverse events associated with corticosteroid therapy were mild and transient. Clinical responses did not correlate with improvements in either intramedullary fibrosis or angiogenesis. THAL-PRED is well tolerated and preliminarily appears to be a promising drug regimen for treating cytopenias in patients with MMM.

PMID 12517815  Blood. 2003 Apr 1;101(7):2534-41. doi: 10.1182/blood-20・・・
著者: Ayalew Tefferi, Jorge Cortes, Srdan Verstovsek, Ruben A Mesa, Deborah Thomas, Terra L Lasho, William J Hogan, Mark R Litzow, Jacob B Allred, Dan Jones, Catriona Byrne, Jerome B Zeldis, Rhett P Ketterling, Rebecca F McClure, Francis Giles, Hagop M Kantarjian
雑誌名: Blood. 2006 Aug 15;108(4):1158-64. doi: 10.1182/blood-2006-02-004572. Epub 2006 Apr 11.
Abstract/Text We present results of 2 similarly designed but separate phase 2 studies involving single-agent lenalidomide (CC-5013, Revlimid) in a total of 68 patients with symptomatic myelofibrosis with myeloid metaplasia (MMM). Protocol treatment consisted of oral lenalidomide at 10 mg/d (5 mg/d if baseline platelet count < 100 x 10(9)/L) for 3 to 4 months with a plan to continue treatment for either 3 or 24 additional months, in case of response. Overall response rates were 22% for anemia, 33% for splenomegaly, and 50% for thrombocytopenia. Response in anemia was deemed impressive in 8 patients whose hemoglobin level normalized from a baseline of either transfusion dependency or hemoglobin level lower than 100 g/L. Additional treatment effects in these patients included resolution of leukoerythroblastosis (4 patients), a decrease in medullary fibrosis and angiogenesis (2 patients), and del(5)(q13q33) cytogenetic remission accompanied by a reduction in JAK2(V617F) mutation burden (1 patient). Grade 3 or 4 adverse events included neutropenia (31%) and thrombocytopenia (19%). We conclude that lenalidomide engenders an intriguing treatment activity in a subset of patients with MMM that includes an unprecedented effect on peripheral blood and bone marrow abnormalities.

PMID 16609064  Blood. 2006 Aug 15;108(4):1158-64. doi: 10.1182/blood-2・・・
著者: Alejandra Martínez-Trillos, Anna Gaya, Margherita Maffioli, Eduardo Arellano-Rodrigo, Xavier Calvo, Marina Díaz-Beyá, Francisco Cervantes
雑誌名: Ann Hematol. 2010 Dec;89(12):1233-7. doi: 10.1007/s00277-010-1019-9. Epub 2010 Jun 22.
Abstract/Text Hydroxyurea (HU) is frequently given as treatment for myelofibrosis (MF), but data on its efficacy and tolerability are scarce. The results of HU therapy were evaluated in 40 patients with hyperproliferative manifestations of primary (n = 32), post-polycythemia vera (n = 6), or post-essential thrombocythemia (n = 2) myelofibrosis. Median interval between diagnosis and HU start was 6.2 months (range 0-141.7). Reasons for treatment were constitutional symptoms (55%), symptomatic splenomegaly (45%), thrombocytosis (40%), leukocytosis (28%), pruritus (10%), and bone pain (8%). The starting dose was 500 mg/day, subsequently adjusted to the individual efficacy and tolerability. Response was bone pain 100%, constitutional symptoms 82%, pruritus 50%, splenomegaly 40%, and anemia 12.5%. According to the International Working Group for Myelofibrosis Research and Treatment criteria, clinical improvement was achieved in 16 patients (40%). Median duration of response was 13.2 months (range 3-126.2). Worsening of the anemia or appearance of pancytopenia were observed in 18 patients, requiring administration of erythropoietin-stimulating agents (n = 17) and/or danazol (n = 9). Oral or leg ulcers appeared in five patients and one had gastrointestinal symptoms. HU is an effective and generally well-tolerated therapy for the hyperproliferative manifestations of MF. The accentuation of the anemia often induced by HU is usually manageable with concomitant treatment.

PMID 20567824  Ann Hematol. 2010 Dec;89(12):1233-7. doi: 10.1007/s0027・・・
著者: M A Elliott, M G Chen, M N Silverstein, A Tefferi
雑誌名: Br J Haematol. 1998 Nov;103(2):505-11.
Abstract/Text Twenty-three patients who had myelofibrosis with myeloid metaplasia (MMM) were treated at our institution with 50 courses of splenic irradiation (SI) for symptomatic splenomegaly. The median dose of radiation per course was 277.5 cGy, administered in a median of 7.5 fractions. 8/23 patients received multiple courses of SI. Of 49 evaluable courses of SI, 46 (93.9%) resulted in an objective decrease in spleen size. The median duration of response was 6 months (range 1-41). Reduction in spleen size was associated with symptomatic relief in all patients. Overall median survival after SI was 22 months. Significant cytopenia occurred in 10 (43.5%) patients, or 16 (32%) of the 50 courses of SI. Prolonged, life-threatening pancytopenia after a single course of SI occurred in six patients (26%), resulting in fatal sepsis or haemorrhage in three (13%). Nine patients underwent subsequent splenectomy: the perioperative mortality rate was 11%. One third of patients experienced postoperative intra-abdominal haemorrhage necessitating surgical re-exploration. SI can provide symptomatic relief and a reduction in spleen size in most MMM patients. The increased risk of postoperative bleeding in patients requiring subsequent splenectomy dictates against considering SI as an alternative to splenectomy for patients who are otherwise good surgical candidates.

PMID 9827926  Br J Haematol. 1998 Nov;103(2):505-11.
著者: P Guardiola, J E Anderson, G Bandini, F Cervantes, V Runde, W Arcese, A Bacigalupo, D Przepiorka, M R O'Donnell, P Polchi, A Buzyn, L Sutton, D Cazals-Hatem, G Sale, T de Witte, H J Deeg, E Gluckman
雑誌名: Blood. 1999 May 1;93(9):2831-8.
Abstract/Text Agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder in which patients with poor prognostic features, receiving conventional treatments, have a median survival of less than 3 years. In this retrospective multicenter study, we analyze the results and try to define the indications for allogeneic stem cell transplantation in AMM. From January 1979 to November 1997, 55 patients with a median age of 42 years were transplanted from HLA-matched related (n = 49) or alternative (n = 6) donors for AMM. A multivariate analysis was conducted to identify factors associated with posttransplant outcome. The median posttransplant follow-up was 36 months (range, 6 to 223). The 5-year probability of survival was 47% +/- 8% for the overall group, and 54% +/- 8% for patients receiving an unmanipulated HLA-matched related transplant. The 1-year probability of transplant-related mortality was 27% +/- 6%. Hemoglobin level
PMID 10216077  Blood. 1999 May 1;93(9):2831-8.

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