Noter
Diasshow
Disposition
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Working Classification of
Chronic Myeloid Disorders
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De Ph-negative
kroniske myeloproliferative sygdomme

  • Erhvervede stamcellesygdomme
  •    (klonale myeloprolifeartive sygdomme)
  • Overgangsformer
  • Thrombohæmorrhagiske komplikationer
  • Myelofibrosis
  • Terminalt knoglemarvssvigt/AML



4
De Ph-negative
kroniske myeloproliferative sygdomme
Behandling

  • Venesektion ( PV) (ht < 0.42 k; < 0.45 m)


  • Cytoreduktion


  • Apurin


  • Acetylsalicylsyre (PV/ET)


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Rate of rise in hematocrit in a patient with polycythemia vera who sought treatment of the disease 9 years after the hematocrit began to increase
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Treatment guidelines for PV and ET Nordic MPD study group
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Idiopathic myelofibrosis



  •    ‘…..….this malady of mystery’.




  •    Murray Silverstein (1975)
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Potential Cell-Cytokine Interactions
 in the Pathogenesis of Myelofibrosis with
 Myeloid Metaplasia
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Symptoms and Clinical Findings

   Early Prefibrotic                                 Advanced                                                Idiopathic Myelofibrosis                 Idiopathic Myelofibrosis

  • Thrombocytosis
  • Hypermetabolic Symptoms
  • Myeloid Metaplasia
  • Bone marrow failure



  • Thrombocytopenia
  • Hypermetabolic Symptoms
  • Myeloid Metaplasia
  • Bone marrow failure
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Symptoms and Clinical Findings

   Early Prefibrotic                                 Advanced                                                Idiopathic Myelofibrosis                 Idiopathic Myelofibrosis

  • Thrombocytosis
  • Hypermetabolic Symptoms
  • Myeloid Metaplasia
  • Bone marrow failure




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Symptoms and Clinical Findings
  • Thrombocytosis


  • Hypermetabolism
  • Bone Marrow Failure
  • Myeloid Metaplasia
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Symptoms of Thrombocytosis 
Early Prefibrotic Idiopathic Myelofibrosis

  • Thrombocytosis - micro/macrocirculatory disturbances /bleeding
  • Leucocytosis
  • No or minimal Anemia
  • No or minimal Splenomegaly
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Idiopathic Myelofibrosis
Early Prefibrotic Myelofibrosis
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Pathological Features of Peripheral Blood and Bone Marrow in Myelofibrosis with Myeloid Metaplasia
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The differential diagnosis of massive splenomegaly, operationally defined as a palpable spleen that extends below the transumbilical line
  • Myelofibrosis with myeloid metaplasia (MMM)
  • Chronic myeloid leukemia
  • Malignant lymphoma
  • Hairy cell leukemia
  • Hyperreactive malarial splenomegaly
  • Kala-azar (visceral leishmaniasis)



  • Gaucher disease
  • Splenic cystic lymphangiomatosis
  • Systemic lupus erythematosus,
  • Sarcoidosis
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The differential diagnosis of massive splenomegaly, operationally defined as a palpable spleen that extends below the transumbilical line
  • Myelofibrosis with myeloid metaplasia (MMM)
  • Chronic myeloid leukemia
  • Malignant lymphoma
  • Hairy cell leukemia
  • Hyperreactive malarial splenomegaly
  • Kala-azar (visceral leishmaniasis)



  • Gaucher disease
  • Splenic cystic lymphangiomatosis
  • Systemic lupus erythematosus,
  • Sarcoidosis
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Modern Treatment of Myelofibrosis With Myeloid Metaplasia
(MMM)
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The Spleen in Myelofibrosis With Myeloid Metaplasia
(MMM)


In MMM splenomegaly is secondary to extramedullary hematopoiesis
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Symptoms of Progressive Splenomegaly in Myelofibrosis with Myeloid Metaplasia
  • Mechanical discomfort
  • Early satiety
  • Episodes of severe left upper quadrant pain from splenic infarcts
  • Profound cachexia
  • Increased red blood cell transfusion requirement
  • Thrombocytopenia
  • Complications of portal hypertension
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Tefferi A, Mesa RA, Nagorney DM, Schroeder G,
Silverstein MN.
Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients. 
Blood. 2000;95:2226-2233.
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Prognosis in Myelofibrosis With Myeloid Metaplasia
(MMM)


In MMM
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  The Lille Scoring System
(adverse prognostic factors Hb<10; WCC<4 >30