OMF und Splenomegalie

[Rüdiger]

Vor 4 Jahren wurde bei mir OMF diagnostiziert. Jetzt leide ich u.a. zunehmend unter der sehr stark vergrößerten Milz. Andererseits sind die Blutwerte größtenteils im Normbereich. Wer ist auch an OMF erkrankt, wer hat entsprechendes Fachwissen und ist zu einem Erfahrungsaustausch bereit?

[Prof. Wust]

RE: OMF und Splenomegalie


Bei der (idiopathischen?) Osteomyelofibrose kann die Milz aufgrund der extramedullären Blutbil-dung sehr vergrößert sein. Allgemein wird dann (mindestens wenn Beschwerden bestehen) die Splenektomie empfohlen. Es gibt sehr wenig Erfahrungen mit dieser Erkrankung, weil sie selten ist. Selbst bei einer Medline Recherche habe ich in den letzten 10 Jahren ganz wenige (klinisch orientierte) Arbeiten gefunden. Eine beschreibt die Splenektomie (s.3.). Diese Arbeit sollten Sie sich besorgen. Die Recherche habe ich zu Ihrer Information angehängt.

1. Myeloproliferative disorders: complications, survival and causes of death.
AU: Brodmann,-S; Passweg,-J-R; Gratwohl,-A; Tichelli,-A; Skoda,-R-C
AD: Biozentrum, University of Basel, Switzerland.
SO: Ann-Hematol. 2000 Jun; 79(6): 312-8
IS: 0939-5555
PY: 2000
LA: English
CP: GERMANY
AB: This retrospective single-center study compared thromboembolic and hemorrhagic compli-cations, survival and causes of death in a cohort of 102 consecutive patients with myeloprolife-rative disorders (MPD). We included 17 patients with essential thrombocythemia (ET), 59 with polycythemia vera (PV), and 26 with osteomyelofibrosis (OMF). The median follow-up was 3.7 years. Estimated 8-year probability of complications for the entire cohort was 80 +/- 11% (95% confidence interval), without significant differences among MPD subgroups. The rate of throm-boembolic complications, expressed as the number of events per 100 patient years, was 16.7 for patients with PV, 13.8 for OMF, and 7.5 for ET. Fifty-four percent of thromboembolic events in PV involved cerebral or limb arteries. The rate of bleeding complications was highest in patients with OMF (31.8 per 100 patient years), followed by ET and PV (11.8). Ninety percent of bleeding episodes affected the skin. mucosal membranes, and the gastrointestinal tract. Eight-year survi-val was highest in ET with 91 +/- 17%, followed by PV (66 +/- 18%) and OMF (40 +/- 31%) (P< 0.01). Twenty-four patients died during the observation period, and fatal thrombosis (in five pati-ents) represented the leading cause of death. Only two patients with MPD died from fatal he-morrhage and one from acute leukemia. We conclude that survival is highest in ET and lowest in OMF. Both thromboembolic and hemorrhagic complications are frequent. However, thrombosis appears to be more often fatal than bleeding complications. Prophylaxis of thromboembolic events remains a key issue in the management of MPD.

2. Prognostic factors in idiopathic (primary) osteomyelofibrosis.
AU: Kvasnicka,-H-M; Thiele,-J; Werden,-C; Zankovich,-R; Diehl,-V; Fischer,-R
AD: Institute of Pathology, University of Cologne, Germany.
SO: Cancer. 1997 Aug 15; 80(4): 708-19
IS: 0008-543X
PY: 1997
LA: English
CP: UNITED-STATES
AB: BACKGROUND: Prognostic variables for idiopathic (primary) osteomyelofibrosis (IMF) are ill-defined because of the lack of large control studies based on uniform diagnostic criteria. ME-THODS: A retrospective clinicopathologic study was performed on 250 consecutively recruited patients (115 males and 135 females) with an established diagnosis of IMF. In contrast to pre-vious studies, the current study cohort encompassed the full spectrum of initial to advanced sta-ges of the disease process according to laboratory data and particularly histology. Because of the relatively high patient age on admission (median, 66.5 years), relative survival rates with corresponding life expectancies and disease specific life loss were calculated. Moreover, a clas-sification and regression tree (CART) analysis was performed to segregate the study patients into subgroups with significantly different prognosis. RESULTS: Analysis of the life expectancy and the proportion of deaths attributable to IMF showed a global reduction in life expectancy of 31%. Further calculation disclosed a consistently greater impact of disease in older patients. Age, hemoglobin level on admission, and leukocyte and thrombocyte counts remained as the most relevant parameters for prognosis in multivariate consideration (CART analysis) and facilitated a clear-cut separation into three risk groups. The life expectancy of low risk patients was approximately 10 times higher than that of high risk patients (22.07 years vs. 2.25 years). CON-CLUSIONS: These results are in keeping with the assumption that features signaling bone mar-row insufficiency are associated with a worsening of survival. Generalization, indicated by mye-loid metaplasia, can occur at every stage, even in so-called hypercellular phases of IMF. Con-versely, myelofibrosis alone is not necessarily predictive of poor survival.

3. Splenektomie bei Osteomyelofibrose. Indikationen und Ergebnisse.
[Splenectomy in osteomyelofibrosis. Indications and outcome]
AU: Bohner,-H; Rotzscher,-V-M; Tirier,-C; Heit,-W; Greiner,-J
AD: Chirurgische Klinik, Elisabeth-Krankenhaus Essen.
SO: Chirurg. 1996 Oct; 67(10): 1016-9
IS: 0009-4722
PY: 1996
LA: German; Non-English
CP: GERMANY
AB: Osteomyelofibrosis is a myeloproliferative disorder in which fibrosis and sclerosis finally lead to bone marrow obliteration. Liver and spleen compensate for bone marrow loss with extrame-dullary hematopoiesis. In some patients the resulting splenomegaly causes severe symptoms such as local compression, thrombocytopenia and hemolytic anemia. In such patients, splenec-tomy is the only promising treatment, although it represents a significant risk.
MESH: *Myelofibrosis-surgery; *Splenomegaly-pathology; *Splenomegaly-radiography; *Splenomegaly-surgery
MESH: Adult-; Aged-; Blood-Coagulation-Tests; Follow-Up-Studies; Middle-Age; Myelofibrosis-pathology; Myelofibrosis-radiography; Platelet-Count; Postoperative-Complications-blood; Post-operative-Complications-mortality; Survival-Rate; Tomography,-X-Ray-Computed; Treatment-Outcome

4. Incidence and clinical risk factors for bleeding and thrombotic complications in myeloproliferative disorders. A retrospective analysis of 260 patients.
AU: Wehmeier,-A; Daum,-I; Jamin,-H; Schneider,-W
AD: Medizinische Klinik, Abteilung fur Hamatologie, Dusseldorf, FRG.
SO: Ann-Hematol. 1991 Aug; 63(2): 101-6
IS: 0939-5555
PY: 1991
LA: English
CP: Index-M
AB: Bleeding and thrombosis are frequent complications in myeloproliferative disorders (MPD) and are associated with severe organ damage and a high mortality. Elevated platelet count, ele-vated hematocrit, and patient age are regarded as risk factors for bleeding and thromboembolic events in MPD, although the significance of these parameters was not confirmed by clinical studies. We retrospectively analyzed vascular complications in 260 patients with MPD and tried to identify parameters predictive for bleeding and thrombembolic events. Our cohort consisted of 115 patients with chronic myeloid leukemia (CML), 84 patients with polycythemia vera (PV), 26 with essential thrombocythemia (ET), 25 with osteomyelofibrosis (OMF), and 10 patients with unclassifiable MPD. During a median follow-up period of 31 months, 126 patients with chronic MPD suffered bleeding or thrombotic events. Bleeding was observed in 57% of patients with OMF, 23% with PV, 20% with chronic phase CML, and 16% with ET. Thrombotic events were most common in patients with PV (36% of patients), followed by ET, OMF, and chronic phase CML (20%, 17%, and 6% of patients, respectively). Recurrent thrombotic episodes frequently occurred in patients with PV and ET, whereas patients with OMF often had more than one blee-ding event. Thirty patients died of thrombohemorrhagic complications during follow-up. Multiva-riate analysis, including all patients with chronic MPD, revealed that elevated red blood cell count, higher hemoglobin level, and increased percentage of segmented neutrophils at the time of diagnosis were associated with thrombosis, whereas patients with bleeding complications were characterized by low red cell count, lower hemoglobin, and a lower percentage of segmented neutrophils. However, when analyzed by MPD subgroup, none of these parameters retained a predictive value for bleeding or thrombotic events. Moreover, elevated platelet count and patient age were not risk factors for bleeding complications. Thrombotic events were less frequent in patients below the age of 40, and were increased in patients aged 70 and above. However, this was primarily due to the high percentage of elderly patients in subgroups mainly affected by thrombosis (PV and ET). In most MPD subgroups, the rate of bleeding and thrombosis was highest just before and during the first months after diagnosis, and declined thereafter. Thrombohemorrhagic complications were less frequent after phlebotomy in PV and after therapy with alkylating agents in CML. The institution of cytoreductive therapy soon after the diagnosis was made may explain the reduced incidence of complications later in the disease. We conclude that morbidity and mortality from thrombohemorrhagic complications are high in myeloproliferative disorders.(ABSTRACT TRUNCATED AT 400 WORDS)
MESH: *Hemorrhage-etiology; *Myeloproliferative-Disorders-complications; *Thrombosis-etiology
MESH: Bloodletting-; Busulfan-therapeutic-use; Cause-of-Death; Hemorrhage-epidemiology; Incidence-; Leukemia,-Myeloid,-Chronic-drug-therapy; Polycythemia-Vera-therapy; Retrospective-Studies; Risk-Factors; Survival-Analysis; Thrombosis-epidemiology
TG: Human
PT: Journal-Article
SH: therapeutic-use; epidemiology; etiology; drug-therapy; complications; therapy
RN: 55-98-1
NM: Busulfan