This month, Spero Cataland, MD, discusses whether a patient with thrombocytopenia has hypersplenism, immune thrombocytopenia, or both.
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I have a 22-year-old Vietnamese female patient who moved to the U.S. at around age 10. She has no pertinent family history. She presented two years ago to an outside hospital with epistaxis and was found to have a platelet count of zero. She was also noted to have abnormal liver function tests. The workup, including a liver biopsy, revealed autoimmune hepatitis with grade 4 cirrhosis. She responded to immune thrombocytopenia purpura (ITP)–directed therapy (steroids, intravenous immunoglobin [IVIG] therapy, and platelet transfusion). She did not follow up regularly, but had at least two subsequent bone marrow biopsies, the second of which showed megakaryocyte hyperplasia. More recently, she presented in a similar way to how she did initially (2 years ago) and was treated the same – steroids, IVIG, and prednisone. She was also given one dose of rituximab. Her platelet count increased to 99×109/L, but is dropping to 77×109/L. What is the best approach to obtain remission? Is splenectomy the next step? She is also being referred for possible liver transplantation, so how does ITP treatment factor into the question of liver transplant? She has no history of alcohol use.
This is certainly a very challenging case that you have on your hands. When I heard cirrhosis and thrombocytopenia, my first thought was splenic sequestration instead of ITP, but her previous complete blood count with a platelet count of 1×109/L would fit with a diagnosis of ITP. Given the recent platelet count of 70 to 90×109/L, I would consider reimaging now to make sure that progressive spleno-megaly and sequestration have not developed since her last acute ITP event. If that is the case, then what you are seeing now may be more consistent with hypersplenism as the cause of her thrombocytopenia.
If hypersplenism doesn’t explain the picture, it may be a recurrence of her ITP that likely is secondary to her autoimmune disease (hepatitis). The rituximab she already received may still be helping, but it may take more time. Her platelet count is not too bad at 70 to 90×109/L, so if there are no issues with bleeding I would consider watching her (as the goals of therapy are to maintain a safe platelet count of 30 to 40×109/L with as little treatment as possible). If she needs treatment to maintain a safe platelet count, I might consider the use of the thrombopoietin (TPO) agents eltrombopag or romiplostim. In many patients with liver disease, the thrombocytopenia may also be a function of TPO deficiency as it is produced in the liver. Splenectomy is typically a very good choice for patients with ITP, but I would not proceed with this in the context of portal hypertension/sequestration from liver disease, if present. This can lead to an increased risk for portal and splenic vein thromboses postoperatively. In addition, if a liver transplant could be in her future, I might want to avoid a splenectomy and first try to manage her ITP (if treatment is needed) with the TPO agents.
NEXT MONTH'S CLINICAL DILEMMA
I have a patient who is a Jehovah’s Witness with stage IV uterine cancer who was admitted to a community hospital with a uterine abscess. Her hemoglobin is low and I am giving her epoetin alfa and intravenous iron. Are there any other options for treatment of anemia for patients who refuse packed red blood cell transfusions? Her son suggested PolyHeme (a human hemoglobin-based red cell substitute). Is it U.S. Food and Drug Administration–approved?
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