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Case Report: We report a case of a young African American female who presented with severe anemia and leukopenia, subsequently diagnosed with myelofibrosis and then eventually SLE. The identification of myelofibrosis in SLE is critical as it can be a devastating condition when untreated.
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Myelofibrosis is a rare condition, with about 1.5 cases reported per 100,000 people each year in the United States. It occurs in both men and women.
Myelofibrosis causes extensive scarring in your bone marrow, leading to severe anemia that can cause weakness and fatigue. Bone marrow scarring can also cause you to have a low number of blood-clotting cells called platelets, which increases the risk of bleeding. Myelofibrosis often causes an enlarged spleen.
The median survival of patients with primary myelofibrosis is approximately 6 years. It has been observed that the natural course of the disease has changed with studies such as allogeneic hematopoietic stem cell transplantation (allo-HSCT).
Blood tests. In myelofibrosis, a complete blood count typically shows abnormally low levels of red blood cells, a sign of anemia common in people with myelofibrosis. White blood cell and platelet counts are usually abnormal, too.
When you have myelofibrosis, your body makes more uric acid than normal. When it builds up, it forms needle-like crystals in your joints. They cause sharp pain, swollen joints, and inflammation.
The drugs fedratinib (Inrebic) pacritinib (Vonjo), and ruxolitinib (Jakafi) are approved to treat MF. Most people with MF have a mutation, or change, in one of their genes that tell their body how to make blood cells. These inhibitors are used to block the processes those faulty genes.
Results from a retrospective study suggest that identification of patients with primary myelofibrosis likely to survive 20+ years can be made on the basis of age, sex, blood counts, and symptoms, without inclusion of genetic risk factors. This study was published in the American Journal of Hematology.
In some people their disease remains stable for long periods and they are free to live a normal life with minimal interruptions from their disease or its treatment. For others, myelofibrosis progresses more quickly and people require treatment to help relieve symptoms of their disease.
A process called allogenic stem cell transplantation is the only cure. In myelofibrosis, your stem cells don't work like they should. This causes scar tissue to build up in your bone marrow. During this treatment, you're given healthy stem cells from a donor to replace your faulty ones.
The U.S. Food and Drug Administration (FDA) has granted marketing approval for the drug pacritinib (Vonjo) for patients with myelofibrosis (MF), a rare blood cancer that leads to impaired production of normal blood cells.
For the myelofibrosis diagnosis, one needs to establish certain criteria: The bone marrow findings, including the presence of fibrosis. Fibrosis is not exclusive for myelofibrosis. It can be seen as reactive and in other hematologic malignancies. There are stages of myelofibrosis where fibrosis can't be seen.
Symptoms of MyelofibrosisFatigue, weakness, shortness of breath, or pale skin because of a low number of red blood cells (anemia)Frequent infections because of a low white blood cell count (neutropenia)Easy bleeding or bruising because of a lack of platelets in your blood (thrombocytopenia)More items...•
Myelofibrosis is a hematologic condition that predisposes to the formation of large and small portal venous clots. Portal injury is believed to underlie the mechanism of development of noncirrhotic portal hypertension in this population.
Myelofibrosis is a neoplastic disorder characterized by progressive fibrotic replacement of the bone marrow, resulting in extramedullary hematopoiesis (EMH). The liver is a well-documented site of EMH.
A 76-year-old woman presented with 1 week of worsening abdominal distension and lower extremity swelling. Examination revealed clear lungs, minimal jugular venous distension, large ascites, splenomegaly without hepatomegaly, and edema.
In myelofibrosis, several disease-specific processes can precipitate portal hypertension. These patients are at risk for hepatic vein thrombosis, and JAK2 positivity specifically raises the specter of Budd-Chiari syndrome.
Author contributions: All authors contributed equally to this manuscript. MS Sherman is the article guarantor.
1. Wanless IR, Peterson P, Das A, Boitnott JK, Moore GW, Bernier V. Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: A clinicopathological study of 145 patients examined at autopsy. Hepatology. 1990;12:1166–74.