You Make the Call
Each month in “ You Make the Call ,” we pick a challenging clinical question submitted through the Consult a Colleague program and post the expert ’ s response , but we also want to know what you would do . Send in your response to next month ’ s clinical dilemma and see how your answer matches up to the expert ’ s in the next print issue .
This month , Spero Cataland , MD , discusses whether a patient with thrombocytopenia has hypersplenism , immune thrombocytopenia , or both .
TRAINING and EDUCATION
Clinical Dilemma :
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 × 10 9 / L , but is dropping to 77 × 10 9 / 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 .
Expert Opinion
Spero Cataland , MD Professor of Internal Medicine Division of Hematology Wexner Medical Center The Ohio State University
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 × 10 9 / L would fit with a diagnosis of ITP . Given the recent platelet count of 70 to 90 × 10 9 / 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 × 10 9 / 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 × 10 9 / 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 hemoglobinbased red cell substitute ). Is it U . S . Food and Drug Administration – approved ?
How would you respond ? Email us at ashclinicalnews @ hematology . org . ●
Consult a Colleague Through ASH
Consult a Colleague is a service for ASH members that helps facilitate the exchange of information between hematologists and their peers . ASH members can seek consultation on clinical cases from qualified experts in 11 categories :
• Anemias
• Hematopoietic cell transplantation
• Hemoglobinopathies
• Hemostasis / thrombosis
• Lymphomas
• Lymphoproliferative disorders
• Leukemias
• Multiple myeloma & Waldenström macroglobulinemia
• Myeloproliferative neoplasms
• Myelodysplastic syndromes
• Thrombocytopenias
Assigned volunteers (“ colleagues ”) will respond to inquiries within two business days ( either by email or phone ).
Have a puzzling clinical dilemma ? Submit a question , and read more about Consult a Colleague volunteers at hematology . org / Clinicians / Consult . aspx or scan the QR code .
* If you have a request related to a hematologic disorder not listed here , please email your recommendation to ashconsult @ hematology . org so it can be considered for addition in the future .
DISCLAIMER : ASH does not recommend or endorse any specific tests , physicians , products , procedures , or opinions , and disclaims any representation , warranty , or guaranty as to the same . Reliance on any information provided in this article is solely at your own risk .
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