Myelofibrosis natural history, complications and prognosis: Difference between revisions

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#[[Platelet]] count <100 × 10<sup>9</sup>/l
#[[Platelet]] count <100 × 10<sup>9</sup>/l
:*The presence of 0, 1, 2 or 3, and ≥4 adverse factors defines low, intermediate-1, intermediate-2 and high-risk [[disease]] with [[median]] survivals of approximately 15.4, 6.5, 2.9 and 1.3 years, respectively.
:*The presence of 0, 1, 2 or 3, and ≥4 adverse factors defines low, intermediate-1, intermediate-2 and high-risk [[disease]] with [[median]] survivals of approximately 15.4, 6.5, 2.9 and 1.3 years, respectively.
{{#Widget:DIPSS_Plus_Score}}
*A [[genetically]] inspired [[Prognosis|prognostic]] scoring system (GIPSS) that stratifies [[Primary myelofibrosis|primary myelofibrosis (PMF)]] [[Patient|patients]] by [[genetic]] variants alone has recently been proposed but the lack of overlapping [[Prognosis|prognostic]] variables between the dynamic international prognostic scoring system (DIPSS) and the genetically inspired prognostic scoring system (GIPSS) has also increased the risk for disagreement between the two valid [[Prognosis|prognostic]] models.<ref name="pmid30390311">{{cite journal |vauthors=Kuykendall AT, Talati C, Padron E, Sweet K, Sallman D, List AF, Lancet JE, Komrokji RS |title=Genetically inspired prognostic scoring system (GIPSS) outperforms dynamic international prognostic scoring system (DIPSS) in myelofibrosis patients |journal=Am. J. Hematol. |volume= |issue= |pages= |date=November 2018 |pmid=30390311 |doi=10.1002/ajh.25335 |url=}}</ref>
*A [[genetically]] inspired [[Prognosis|prognostic]] scoring system (GIPSS) that stratifies [[Primary myelofibrosis|primary myelofibrosis (PMF)]] [[Patient|patients]] by [[genetic]] variants alone has recently been proposed but the lack of overlapping [[Prognosis|prognostic]] variables between the dynamic international prognostic scoring system (DIPSS) and the genetically inspired prognostic scoring system (GIPSS) has also increased the risk for disagreement between the two valid [[Prognosis|prognostic]] models.<ref name="pmid30390311">{{cite journal |vauthors=Kuykendall AT, Talati C, Padron E, Sweet K, Sallman D, List AF, Lancet JE, Komrokji RS |title=Genetically inspired prognostic scoring system (GIPSS) outperforms dynamic international prognostic scoring system (DIPSS) in myelofibrosis patients |journal=Am. J. Hematol. |volume= |issue= |pages= |date=November 2018 |pmid=30390311 |doi=10.1002/ajh.25335 |url=}}</ref>
*The [[Genetics|genetically]] inspired prognostic scoring system (GIPSS) performs equally well for both primary and secondary [[myelofibrosis]] and outperforms the dynamic international prognostic scoring system (DIPSS) in [[Patient|patients]] where the two models disagree.
*The [[Genetics|genetically]] inspired prognostic scoring system (GIPSS) performs equally well for both primary and secondary [[myelofibrosis]] and outperforms the dynamic international prognostic scoring system (DIPSS) in [[Patient|patients]] where the two models disagree.

Revision as of 20:45, 3 January 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]

Overview

The development of myelofibrosis is a a slow process and it does not cause early symptoms. A significant proportion of the patients can be asymptomatic and the diagnosis is usually made in the setting of an unrelated condition. The most overlapping and common findings encountered are anemia and splenomegaly presenting as weakness, easy fatigability, palpitations, and dyspnea in the case of anemia and early satiety with possible accompanying left upper quadrant discomfort if splenomegaly is present.

The disease has a progressive course and can result in pancytopenia as the bone marrow failure ensues. This can result in bleeding complications, easy bruising, increase in the susceptibility to infections, and worsening anemia. The bone marrow failure paves the way for extramedullary hematopoiesis (EMH) which mainly occurs in the reticuloendothelial tissues.

If left untreated, myelofibrosis can lead to severe complications, the most feared of which are acute leukemia, heart failure, and portal hypertension.

Natural History

Complications

Common complications of myelofibrosis include:

Prognosis

  1. Age >65 years
  2. Hemoglobin <10 g/dl
  3. Leucocyte count >25 × 109/l
  4. Circulating blasts ≥1%
  5. Constitutional symptoms
  6. Unfavourable karyotype (i.e., complex karyotype or sole or two abnormalities that include +8, -7/7q-, i(17q), inv(3), 5/5q-, 12p-, or 11q23 rearrangement)
  7. Transfusion dependency
  8. Platelet count <100 × 109/l
  • The presence of 0, 1, 2 or 3, and ≥4 adverse factors defines low, intermediate-1, intermediate-2 and high-risk disease with median survivals of approximately 15.4, 6.5, 2.9 and 1.3 years, respectively.

DIPSS Plus Score for Prognosis in Myelofibrosis
Variable Score
Age >65 years 1
Constitutional symptoms 1
Hemoglobin <10 g/dL 1
Leucocyte count >25 x 10⁹/L 1
Circulating blasts ≥1% 1
Unfavourable karyotype (complex karyotype or abnormalities that include +8, -7/7q-, i(17q), inv(3), 5/5q-, 12p-, or 11q23 rearrangement) 1
Transfusion dependency 1
Platelet count <100 x 10⁹/L 1
DIPSS Plus Score:
Interpretation:


References

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