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Myeloid Metaplasia


Presentation

  • The interval from MMM diagnosis to presentation with one of the PH features ranged from 1 to 11 years. Variceal bleeding and ascites were the most common presentations.[ncbi.nlm.nih.gov]
  • The spleen is frequently the site of myeloid metaplasia, and it may present with varying degrees of splenomegaly; however, no cases have been reported where this entity presented as an isolated splenic mass.[ncbi.nlm.nih.gov]
  • CIM, presenting as refractory ascites secondary to peritoneal hematopoesis, is extremely rare with only six adult cases reported in literature.[ncbi.nlm.nih.gov]
  • Three infants, including two siblings, presented at ages 9, 10, and 16 months with clinical and laboratory findings consistent with agnogenic myeloid metaplasia.[ncbi.nlm.nih.gov]
  • A review of the literature and an analysis of mechanisms of ascites occurring in AMM, especially peritoneal implants of myeloid tissue and occurrence of peliosis in myeloproliferative disorders, are presented.[ncbi.nlm.nih.gov]
Weight Gain
  • Somnolence, gastro-intestinal signs, weight gain, and edema were significantly more frequent in the thalidomide group.[ncbi.nlm.nih.gov]
Pseudotumor
  • Gastric myeloid metaplasia often has a pseudotumoral appearance, leading to digestive symptoms. Histologic diagnosis is straightforward when trilinear hematopoietic elements are identified in gastric biopsies.[ncbi.nlm.nih.gov]
Long Arm
  • Among cytogenetic studies of patients affected with myelofibrosis with myeloid metaplasia (MMM), a rare chronic myeloproliferative disorder, we found several reports of structural abnormalities of the long arm of chromosome 12.[ncbi.nlm.nih.gov]
  • The remaining 37 patients (46%) demonstrated either interstitial deletions involving the long arm of chromosome 13 or 20 (9 patients; Group 2) or other abnormalities (28 patients; Group 3).[ncbi.nlm.nih.gov]
Musculoskeletal Pain
  • Treatment was held in 16 patients (70%), after 1 to 12 weeks, because of side effects (neutropenia, 6 patients; musculoskeletal pain, 5 patients; thrombocytosis, 4 patients; edema, 3 patients; diarrhea and hyperbilirubinemia, 1 patient).[ncbi.nlm.nih.gov]
Skin Lesion
  • Myeloid metaplasia (lymph nodes and skin lesions) was greatly reduced. The patient experienced no side effects. We propose further trials to confirm the utility of this agent in this disease.[ncbi.nlm.nih.gov]
Hirsutism
  • CASE: An 18-year-old woman was admitted to our clinic due to primary amenorrhea. the On examination she had significant facial hirsutism, clitoromegaly and and Adam's apple. The serum level of testosterone was elevated.[ncbi.nlm.nih.gov]
Ulcer
  • They may present in several ways: erythematous plaques, nodules, diffuse or papular erythema, ulcers, and bullae.[ncbi.nlm.nih.gov]
Withdrawn
  • Occasionally, the spleen reaches giant proportions and must be withdrawn. However, this procedure is followed by greater morbidity and mortality.[ncbi.nlm.nih.gov]
Clitoromegaly
  • CASE: An 18-year-old woman was admitted to our clinic due to primary amenorrhea. the On examination she had significant facial hirsutism, clitoromegaly and and Adam's apple. The serum level of testosterone was elevated.[ncbi.nlm.nih.gov]

Workup

  • The workup of these unusual extramedullary myeloproliferative masses was aided when immunocytochemistry and EM were performed on the aspirated material.[ncbi.nlm.nih.gov]
  • Polycythemia Vera Workup. Medscape Drugs and Diseases. Available online at . Accessed on 1/22/17. Lal, A. (2016 November 10 Updated). Essential Thrombocytosis Workup. Medscape Drugs and Diseases. Available online at .[labtestsonline.it]
Pericardial Effusion
  • A pericardial effusion was evident and a pericardial biopsy was consistent with extramedullary hematopoiesis.[ncbi.nlm.nih.gov]
Microcytosis
  • Abstract Microcytosis is a characteristic laboratory feature for both iron deficiency anemia and thalassemia.[ncbi.nlm.nih.gov]

Treatment

  • 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.[ncbi.nlm.nih.gov]
  • Nine patients (45%) showed a favourable response to treatment, including four CR and five PR, four of whom have maintained their response at a median follow-up of 12.5 months (range: 4-21 months) from the start of treatment.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • Univariate analysis indicated that older age was the only factor associated with a favourable response to treatment (P 0.006). None of the patients with appropriate serum erythropoietin levels responded. Treatment was usually well tolerated.[ncbi.nlm.nih.gov]
  • Durable treatment responses were documented for only anemia and thrombocytopenia.[ncbi.nlm.nih.gov]

Prognosis

  • Patients with specific abnormal karyotypes have a poor prognosis and may fail to respond to androgen therapy.[ncbi.nlm.nih.gov]
  • The pathogenesis, clinical characteristics, treatment and prognosis of this condition are discussed.[ncbi.nlm.nih.gov]
  • In conclusion, JAK2(V617F) is a myeloid lineage-specific event, its incidence in MMM is significantly higher with an antecedent history of polycythaemia vera (PV), and its presence in AMM does not affect prognosis but is associated with PV-characteristic[ncbi.nlm.nih.gov]
  • Prognosis Survival 5 years, often progresses to acute leukaemia. Terminology No name used for this condition has proven consistently satisfactory to those who work in the field.[medical-dictionary.thefreedictionary.com]

Etiology

  • ., the formation of bone marrow elements outside of the medullary cavity, may occur in any patient with anemia, regardless of the etiology of the anemia itself.[ncbi.nlm.nih.gov]
  • As in other etiologies, variceal bleeding and ascites are the most common presentations. Anemia may correlate with, and/or predict, the severity of the PH presentation in these patients. EVL can successfully control variceal bleeding in MMM.[ncbi.nlm.nih.gov]
  • FGF from megakaryocytes. 3) A statistically significant correlation between the growth factor mRNA levels with the degree of myelofibrosis in AMM suggests that these fibrosing growth factors produced by the megakaryocytes may be associated with the etiology[ncbi.nlm.nih.gov]

Epidemiology

  • Epidemiological data are not available. Mortality/Morbidity: MF causes, or accompanies conditions that cause, disruption of hematopoiesis. Patients may experience anaemia, neutropenia, and/or thrombocytopaenia.[myvmc.com]
  • […] permission PEIR, University of Alabama at Birmingham, Department of Pathology Myelofibrosis Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Myelofibrosis from other Diseases Epidemiology[wikidoc.org]
  • Epidemiology MMM has an incidence of 0.3 to 1.5 new cases per year in 100.000 persons. Male predominance was observed in some studies and not confirmed in others. The average age at diagnosis is 60 years.[atlasgeneticsoncology.org]
  • Epidemiology Incidence Although this condition is seen at any age, it is most often seen over the age of 50 years. Peak incidence is at 60-70 years. Prevalence It is very rare. Approximately 1 case per 300,000 per year.[patient.info]
  • If the patient is diabetic and is taking sulfonylureas medication this should be stopped periodically to rule out drug-induced thrombocytopenia . [ citation needed ] [2] Epidemiology [ edit ] The disorder usually develops slowly and is mainly observed[en.wikiversity.org]
Sex distribution
Age distribution

Pathophysiology

  • Recent advances into the pathophysiology of the disease have not yet translated into effective therapeutic options for patients.[ncbi.nlm.nih.gov]
  • Given the importance of core-binding factor in megakaryocytic differentiation and platelet production, as well as the central role of megakaryocytes in the pathophysiology of myelofibrosis with myeloid metaplasia (MMM), we hypothesised that RUNX1 gene[ncbi.nlm.nih.gov]
  • Abstract Angiogenesis is part of the pathophysiology of myelofibrosis with myeloid metaplasia (MMM). PTK787/ZK 222584 (PTK/ZK) is a novel inhibitor of vascular endothelial growth factor receptors.[ncbi.nlm.nih.gov]
  • Abstract Myeloproliferation, myelofibrosis, and neoangiogenesis are the 3 major intrinsic pathophysiologic features of myeloid metaplasia with myelofibrosis (MMM).[ncbi.nlm.nih.gov]
  • Image courtesy of Professor Peter Anderson DVM PhD and published with permission PEIR, University of Alabama at Birmingham, Department of Pathology Myelofibrosis Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology[wikidoc.org]

Prevention

  • Whether IFN-alpha therapy can prevent the progression of idiopathic myelofibrosis to leukemia has not yet been determined.[ncbi.nlm.nih.gov]
  • Early recognition of pulmonary EMH may prevent PH and favor a better response to therapy. 2006 Wiley-Liss, Inc.[ncbi.nlm.nih.gov]
  • Remedies Agnogenic Myeloid Metaplasia – Causes, Symptoms, Diagnosis, Prevention, Treatment, and Remedies[theteamwork.com]
  • Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case # 1 Related chapters Myelophthisic anemia[wikidoc.org]
  • We don’t know how to prevent MF. But there’s research under way to find out more about it. Who Gets MF? Most people are diagnosed around age 60. Both men and women can get it. About 18,000 people in the U.S. are living with MF.[webmd.com]

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