Anemia In Myelophthise

The anemia in Myelophthise is a normochromic normocytic anemia, which is characterized by the infiltration or displacement of the normal bone marrow by nichthämatopoetische or abnormal cells. It can be caused by tumor cells by granulomatous disease or lipid storage diseases. Frequently a bone marrow fibrosis occurs. In addition, splenomegaly may develop. The typical changes in the peripheral blood include anisocytosis, poikilocytosis and the occurrence of erythrocyte and leukocyte progenitors (leukoerythroblastisches blood count). To help diagnose a bone marrow biopsy is usually required. Treatment consists of supportive measures, which are based on the underlying disease.

The various terms used to describe this form of anemia can be confusing. In myelofibrosis, a replacement of the bone marrow by fibrous connective tissue strands is present. It can be idiopathic (primary) or be secondary. In a real myelofibrosis is a stem cell defect in which the fibrosis occurs secondary to other hematopoietic intramedullary events. Under myelosclerosis is the formation of new bone tissue that accompanies myelofibrosis occasionally. The term myeloid metaplasia refers to extramedullary hematopoiesis in the liver, spleen or lymph nodes and can occur together with a Myelophthise any origin. The old name agnogenic myeloid metaplasia refers to a primary myelofibrosis with or without accompanying myeloid metaplasia. (Editor’s note: This term no longer appears today and does not appear in the WHO classification!)

The anemia in Myelophthise is a normochromic normocytic anemia, which is characterized by the infiltration or displacement of the normal bone marrow by nichthämatopoetische or abnormal cells. It can be caused by tumor cells by granulomatous disease or lipid storage diseases. Frequently a bone marrow fibrosis occurs. In addition, splenomegaly may develop. The typical changes in the peripheral blood include anisocytosis, poikilocytosis and the occurrence of erythrocyte and leukocyte progenitors (leukoerythroblastisches blood count). To help diagnose a bone marrow biopsy is usually required. Treatment consists of supportive measures, which are based on the underlying disease. The various terms used to describe this form of anemia can be confusing. In myelofibrosis, a replacement of the bone marrow by fibrous connective tissue strands is present. It can be idiopathic (primary) or be secondary. In a real myelofibrosis is a stem cell defect in which the fibrosis occurs secondary to other hematopoietic intramedullary events. Under myelosclerosis is the formation of new bone tissue that accompanies myelofibrosis occasionally. The term myeloid metaplasia refers to extramedullary hematopoiesis in the liver, spleen or lymph nodes and can occur together with a Myelophthise any origin. The old name agnogenic myeloid metaplasia refers to a primary myelofibrosis with or without accompanying myeloid metaplasia. (Editor’s note: This term no longer appears and does not appear in the WHO classification!) Etiology The most common cause is the displacement of the bone marrow by metastases (most frequently from cancer of the breast and prostate, less frequent kidney, lung, adrenal gland or thyroid). Extramedullary hematopoiesis is usually only moderate. Other reasons include chronic myeloproliferative neoplasms (especially myelofibrosis, polycythemia vera and essential Thrombozythämieim late or end stage), granulomatous diseases and (lipid) storage diseases. Myelofibrosis can occur in all of the aforementioned diseases. Both a reduced amount of functional hematopoietic tissue and by the underlying disease-related metabolic disorders may contribute to decreased erythropoiesis, in some cases, a erythrophagocytosis. The extramedullary hematopoiesis or the opening of the bone marrow sinus lead to the washout of immature cells. Abnormally shaped red blood cells often lead to increased erythrocyte degradation. Symptoms and complaints The myeloid metaplasia may have splenomegaly result, especially in patients with storage diseases. In severe cases, symptoms of anemia and the underlying disorder may be present. A significant splenomegaly may cause abdominal pressure sensation early satiety and abdominal pain in the left upper quadrant. It can be accompanied by hepatomegaly. For a conditional by malignant tumors myelofibrosis hepatosplenomegaly occurs rarely. Diagnostic blood count, erythrocyte indices, reticulocyte and peripheral blood smear bone marrow examination Anemia in Myelophthise should be given in patients with anemia normozytärer into consideration, especially if parallel splenomegaly or primary disease is present, which can lead to this disease. When in doubt, peripheral blood smears, should be established as a leukoerythroblastisches blood count (immature myeloid cells and nucleated red blood cells, usually Normoblasts) suggests the diagnosis of anemia in Myelophthise. Anemia is usually only moderate in severity and typically normocytic to geringgradig macrocytic. The erythrocyte morphology arises in terms of size and shape is very heterogeneous (Anisozytose and poikilocytosis). The white blood cell count can vary. Platelets are often large and have an unusually form, the platelet count is usually reduced. Often occurs reticulocytosis, which can be caused by the premature release of reticulocytes from the bone marrow or regions of extramedullary hematopoiesis. Therefore, a Reticulocytosis not always indicative of increased erythropoiesis. Although peripheral smears may indicate the diagnosis already, a bone marrow examination is usually required. The indication to this is when a leukoerythroblastischen appearance and unclear splenomegaly. The bone marrow aspiration is often difficult and usually a biopsy must be performed. The results are determined by the underlying disorder. Erythropoiesis may be normal or even increased in some cases. Nevertheless, the erythrocyte life is often reduced. Often extramedullary hematopoiesis in the spleen or liver can be detected. Radiographs may show bone lesions (myelosclerosis), which for a longstanding myelofibrosis or other bone changes (z. B. osteoblastic or osteolytic lesions in tumor diseases) are characteristic, and thus indicative of the present kind of anemia. Therapy treatment of the underlying disorder. Transfusions and corticosteroids hydroxyurea (Editor’s note: and JAK inhibitors) may thalidomide The focus is on treating the underlying disorder. In idiopathic cases a purely supportive treatment is carried out. Erythropoietin (20,000 to 40,000 IU of s.c. 1 to 2 times per week) and corticosteroids are used occasionally (eg. Prednisone 10-30 mg po 1 times a day), but so moderate therapeutic success could only be observed. Hydroxyurea (500 mg po 1 times daily or every second day) results in a reduction in spleen size and normalizes erythrocyte levels in many patients, however, pass by the response to treatment often 6 to 12 months. Thalidomide (50-100 mg po 1 time daily in the evening) is likely to result in only a moderate response, but increases the risk of thrombosis and may have severe fatigue result. (Editor’s note: Thalidomide is rarely used Approved the tyrosine kinase inhibitor ruxolitinib is in symptomatic splenomegaly and incidence of severe general symptoms Dosage 5-20 mg po 2 times daily, dose reduction in drop in platelet and white blood cell count…) Summary The anemia in Myelophthise is a normochromic normocytic anemia, which is characterized by the infiltration or displacement of the normal bone marrow by nichthämatopoetische or abnormal cells. The most common cause is the displacement of the bone marrow by metastases, other causes are myeloproliferative diseases, granulomatous diseases and lipid storage diseases. The anemia in Myelophthise should be given in patients with anemia normozytärer and characteristic findings in the peripheral smear into consideration, especially when a splenomegaly or a known underlying disease is present; Confirmation by bone marrow examination. Treatment of the cause and transfusion as needed; Erythropoietin, corticosteroids, hydroxyurea, tyrosine kinase inhibitors; Thalidomide is likely to have the least benefit, but has drawbacks.

Health Life Media Team

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