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]

• Splenomegaly

  Abstract Agnogenic myeloid metaplasia is a chronic myeloproliferative disorder characterized by splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, teardrop-shaped red blood cells, and fibrosis of the bone marrow. [ncbi.nlm.nih.gov]

  Clinical findings Patients are often > age 50, suffer from insidious weight loss, anaemia, and abdominal discomfort due to splenomegaly, often with hepatomegaly; 80% have nonspecific chromosome defects. Diagnosis Bone marrow biopsy. [medical-dictionary.thefreedictionary.com]

• Constitutional Symptom

  Current pharmacologic therapy has been beneficial mainly in terms of palliating disease-associated cytopenias, constitutional symptoms, splenomegaly, and other organ damage from excess myeloproliferation. [ncbi.nlm.nih.gov]

• Fever

  He was hospitalized for neutropenic fever and was diagnosed to have pulmonary aspergillosis. He died of multisystem failure 8 1/2 months after being diagnosed with AMM. [ncbi.nlm.nih.gov]

  Other symptoms may include: Fatigue A general feeling of discomfort Difficulty breathing Weight loss Fever and night sweats Anemia Abnormal bleeding Patients with myelofibrosis have an increased risk of bleeding. [drfarrahcancercenter.com]

  Determine the benefit of this regimen in palliating four hypercatabolic constitutional symptoms (i.e., weight loss, fatigue, drenching night sweats, and unexplained fevers) in these patients. [clinicaltrials.gov]

  But in the course of cell degeneration the body presents the signs and symptoms such as: Bruising Bleeding Pale skin Anemia, fatigue Fever and bone pain Shortness of breath Increased rate of infections Pain below the ribs mainly on the left side Other [gleneaglesglobalhospitals.com]

  Bone pain, fever and night sweats are also common. Peripheral Blood Findings: Moderate to severe anemia with anisocytosis and pronounced poikilocytosis characterized by teardrop shapes (dacryocytes). Nucleated red cells are usually present. [med-ed.virginia.edu]

• Asymptomatic

  Associated disorders include asymptomatic interstitial extramedullary hematopoiesis, obstructive uropathy and nephrolithiasis. Currently, there are no descriptions of the nephrotic syndrome associated with AMM. [ncbi.nlm.nih.gov]

  The first 2 years are usually asymptomatic. [patient.info]

  About a fourth of the patients are asymptomatic and seek medical attention solely because an enlarged spleen is found on routine physical examination, or because of an abnormal peripheral blood smear. [clicktocurecancer.info]

• Severe Pain

  […] complications caused by myelofibrosis are; Gout Bleeding Severe pain Acute leukemia Pain near joints and bones Abnormal growths in other parts of body For diagnosing Myelofibrosis, your doctor performs a physical examination to check the enlargement [gleneaglesglobalhospitals.com]

  (Four-color version of figure on CD-ROM) Splenomegaly in MMM may be complicated by infarction, an event heralded by moderate to severe pain that may be referred to the left shoulder and often requires opiate analgesics to control. 125 CT imaging in such [ncbi.nlm.nih.gov]

  Splenic infarction may occur and results in an acute or subacute onset of severe pain in the left upper quadrant that may be associated with nausea, fever, and referred left shoulder discomfort. [emedicine.medscape.com]

• Pallor

  Bone pain Bruising and easy bleeding due to inadequate numbers of platelets Fatigue Increased susceptibility to infection, such as pneumonia or diarrhea Pallor and shortness of breath while doing physical work due to anemia Because of a high rate of cell [en.wikiversity.org]

  Bruising and easy bleeding due to inadequate numbers of platelets Cachexia (loss of appetite, weight loss, and fatigue) Enlargement of both the liver and spleen Fatigue Gout and high uric acid levels Increased susceptibility to infection, such as pneumonia Pallor [en.wikipedia.org]

• Hepatomegaly

  OBJECTIVE: Therapeutic splenectomy in myelofibrosis with myeloid metaplasia (MMM) may result in extreme thrombocytosis and leukocytosis and accelerated hepatomegaly. [ncbi.nlm.nih.gov]

  Clinical findings Patients are often > age 50, suffer from insidious weight loss, anaemia, and abdominal discomfort due to splenomegaly, often with hepatomegaly; 80% have nonspecific chromosome defects. Diagnosis Bone marrow biopsy. [medical-dictionary.thefreedictionary.com]

  Signs Massive splenomegaly Portal hypertension and hepatomegaly Infections Hyperuricaemia (may show as kidney stones)/ gout Cachexia Amyloidosis Differential diagnosis Metastatic neoplasia Inflammation Leukaemias Chronic myelogenous leukaemia Hairy cell [patient.info]

• Hepatosplenomegaly

  Both had severe anemia, hepatosplenomegaly and bone marrow fibrosis. Lymph node biopsy of the first patient and liver biopsy of the second revealed extramedullary hematopoiesis. [ncbi.nlm.nih.gov]

  Patients may also experience pain secondary to hepatosplenomegaly. Neutropenia may lead to opportunistic infections, such as bacterial sepsis, oral thrush, or systemic fungal infections. Thrombocytopenia may lead to hemorrhage. [myvmc.com]

  Hepatosplenomegaly is rare with myelofibrosis due to malignant tumors. [merckmanuals.com]

• Jaundice

  Side effects of hydroxyurea include myelosuppression, macrocytosis, leg ulcers, increased creatinine level, and jaundice. [clevelandclinicmeded.com]

• Arthritis

  Here is a rare case of myelofibrosis occurring in a patient with juvenile rheumatoid arthritis. [ncbi.nlm.nih.gov]

  化膿性関節炎：suppurative arthritis 結核性関節炎：tuberculous arthritis ◎痛風性関節炎：gouty arthritis 偽痛風：pseudogout ◎色素性絨毛結節性滑膜炎：pigmented villonodular synovitis 腱滑膜炎：tendosynovitis 結節性腱滑膜炎：nodular tendosynovitis ◎腱鞘巨細胞腫：giant cell tumor of tendon sheath ガングリオン：ganglion [medical-e.net]

• Confusion

  The initial symptoms of myelofibrosis may be subtle and are easily confused with the symptoms of many other disorders. [schmidtandclark.com]

  Thus, unless a marrow examination is performed when the patient is initially seen, the clinical diagnosis may be confused with ET. [clicktocurecancer.info]

  Descriptive terms used in this anemia can be confusing. Myelofibrosis, which is replacement of marrow by fibrous tissue bands, may be idiopathic (primary) or secondary. [merckmanuals.com]

  Small mononuclear megakaryocytes have been observed that may be confused with atypical lymphocytes but can be defined due to characteristic demarcation membranes and bullseye granules. [alpfmedical.info]

  Patients with another myeloproliferative neoplasm, essential thrombocythemia, can be confused at times with early stages of PMF, since both conditions are associated with thrombocytosis. [cancertherapyadvisor.com]

• Stroke

  Thrombosis in larger vessels can lead to myocardial infarction, deep venous thrombosis, transient ischemic attacks, and stroke. [clevelandclinicmeded.com]

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 http://emedicine.medscape.com/article/205114-workup#c5. Accessed on 1/22/17. Lal, A. (2016 November 10 Updated). Essential Thrombocytosis Workup. Medscape Drugs and Diseases. [labtestsonline.it]

(See Presentation, Workup, and Treatment .) Splenic infarction may occur and results in an acute or subacute onset of severe pain in the left upper quadrant that may be associated with nausea, fever, and referred left shoulder discomfort. [emedicine.medscape.com]

Of patients with Budd-Chiari syndrome (hepatic-inferior vena cava obstruction), 10% to 13% have coexisting PV; therefore, testing for the presence of JAK2 V617F mutation is part of the routine workup for unexplained liver thrombosis. [clevelandclinicmeded.com]

• Enlargement of the Liver

  It causes extensive scarring of your bone marrow leading to anemia, severe fatigue, and in most cases, an enlarged spleen and liver. Agnogenic Myeloid Metaplasia is same as Myelofibrosis. [theteamwork.com]

• Liver Biopsy

  Lymph node biopsy of the first patient and liver biopsy of the second revealed extramedullary hematopoiesis. [ncbi.nlm.nih.gov]

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]

The pathogenesis, clinical characteristics, treatment and prognosis of this condition are discussed. [ncbi.nlm.nih.gov]

Prognosis is poor, with slow progression and death usually within 2-3 years. [radiopaedia.org]

Myeloid metaplasia, i.e., 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]

[…] the underlying etiology. [icd10data.com]

The absence of involvement of adjacent structures such as bones and the lack of mediastinal inflammation along with the lack of adenopathies made infectious etiologies unlikely. [elynsgroup.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]

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]

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]

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]

Recent advances into the pathophysiology of the disease have not yet translated into effective therapeutic options for patients. [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]

Remedies Agnogenic Myeloid Metaplasia – Causes, Symptoms, Diagnosis, Prevention, Treatment, and Remedies [theteamwork.com]

Whether IFN-alpha therapy can prevent the progression of idiopathic myelofibrosis to leukemia has not yet been determined. [ncbi.nlm.nih.gov]

| 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]