Sarcoidosis is the result of an overactive immune system that damages the body instead of protecting it. Specifically, it leads to the formation of granulomas, which are clumps of inflammatory cells that form within various organs. The accumulation of these granulomas within an organ can impair its proper function.
Sarcoidosis most commonly affects the lungs and the lymphoid system. Sarcoidosis of the spleen has been reported in 24 to 34 percent of cases, while sarcoidosis of the bone marrow affects about 10 percent of patients.
What is sarcoidosis of the spleen and bone marrow?
Bone marrow is the spongy tissue within the bones where blood-forming cells are made. These cells give rise to white blood cells that help fight infection and red blood cells that transport oxygen.
The spleen is a large organ that produces and filters red blood cells and some white blood cells from circulation. In some disorders that disrupt the regular production of blood cells by the bone marrow, the spleen can compensate by producing the blood cells itself.
Sarcoidosis can impair the function of both the spleen and bone marrow affecting blood production.
Symptoms
Involvement of the spleen or bone marrow in sarcoidosis may lead to anemia and other blood abnormalities such as leukopenia and thrombocytopenia.
- Anemia occurs when there aren’t enough red blood cells to carry oxygen throughout the body efficiently.
- Leukopenia, or low white blood cell count, makes people more susceptible to infections.
- Thrombocytopenia, or a deficiency in the number of platelets in the circulation, can lead to excessive bleeding.
Other symptoms of sarcoidosis of the spleen and bone marrow include splenomegaly (enlarged spleen), fatigue, weight loss, fever, night sweats, and pain or pressure under the left ribs where the spleen is located.
Diagnosis
Early diagnosis is vital for any form of sarcoidosis to help prevent organ failure. However, because sarcoidosis can involve many different organs, symptoms are often variable, making it more difficult to diagnose.
Doctors usually begin with basic exams and blood tests, including white and red blood cell counts, along with platelet counts. If a patient shows signs of low blood cell counts, the doctor may perform a bone marrow biopsy to look for granulomas indicative of sarcoidosis.
Doctors will also use their hands to feel the patient’s spleen to see if it is enlarged. They may also perform a computed tomography (CT) or magnetic resonance imaging (MRI) scan of the area around the spleen to confirm the diagnosis.
Doctors must complete a series of tests to rule out more common causes of an enlarged spleen such as viruses or bacteria. Since pathohistological evidence is the most important criterion for diagnosis, doctors may perform a biopsy of the spleen to look for histological evidence of granulomas. They will complete a series of stains and cultures to differentiate sarcoidosis granulomas from other diseases that may cause similar symptoms.
Treatment
Currently, sarcoidosis has no known cure. The first line of treatment for patients with sarcoidosis is corticosteroids, which reduce inflammation associated with sarcoidosis and can prevent further damage to the organs.
In patients with sarcoidosis of the spleen and bone marrow, doctors may prescribe medicines that normalize blood cell counts.
Immunosuppressant agents can be used to suppress the immune system in order to reduce inflammation and prevent further organ damage.
In severe cases, a splenectomy, or removal of the spleen, may be done to ease the pain.
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