This month, Ariela Noy, MD, discusses whether hypogammaglobulinemia alone is an indication for treatment of CLL.
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I have a patient with newly diagnosed chronic lymphocytic leukemia (CLL) who has normal cytogenetics. The presenting features were fever and hypoxia that led to 2 weeks of hospitalization; bronchoscopy revealed pneumocystis pneumonia. The patient is HIV negative and is now doing well without any typical criteria for CLL treatment. He has received intravenous immune globulin (IVIG) twice for IgG level of 200 mg/dL. Is hypogammaglobulinemia alone an indication for treatment for CLL, in the absence of other symptoms or cytopenias?
I see the patient’s infectious complications as having two parts: hypogammaglobulinemia and pneumocystis.
I find the case somewhat puzzling, as pneumocystis has more to do with T cell deficits than IgG status. I found a few case reports of treatment-naïve CLL patients who were diagnosed with pneumocystis,1,2 but it is very rare and more often a complication of treatment with purine analogues, such as fludarabine. In the case reports, lung parenchymal infiltration may have contributed to mechanistic interference of immune response to pneumocystis. Regardless, I recommend determining the CD4 count and, if low, following them over time. Reconstitution will be necessary before discontinuation of any posttreatment pneumocystis prophylaxis.
Hypogammaglobulinemia is quite common in CLL; roughly 25% of patients present with it at diagnosis, and 25% develop it with time.3 Typically, one does not treat with ongoing IVIG on that basis alone unless there are repeated viral or bacterial infections. It is not clear the hypogammaglobulinemia contributed to the pneumocystis. Given the patient’s unusual presentation, I would follow more closely than for asymptomatic CLL.
- Strich JR, Jerussi TD, Wiestner A, Holland, SM. Pneumocystis jirovecii pneumonia in a treatment-naive patient with chronic lymphocytic leukemia. Infect Dis Clin Pract (Baltim Md). 2016;24:e86–e87.
- Kalkanis A, Judson M, Napier M. Pneumocystis jirovecii pneumonia in a patient with untreated chronic lymphocytic leukaemia: a novel case and postulations concerning the mechanism. BMJ Case Rep. 2013;bcr2013202124.
- Parikh SA, Leis JF, Chaffee KG, et al. Hypogammaglobulinemia in newly diagnosed chronic lymphocytic leukemia: natural history, clinical correlates, and outcomes. Cancer. 2015;121:2883-2891.
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NEXT MONTH'S CLINICAL DILEMMA
I am caring for a 33-year-old female patient with JAK2-positive essential thrombocythemia (ET). Her platelet count has been in the range of 800 to 1,000 per mcL. She was initially treated elsewhere with aspirin alone (I understand that acquired von Willebrand disease was excluded) and has since relocated to my community. She was admitted to a hospital with injuries from a motor vehicle accident secondary to polysubstance abuse and required a splenectomy earlier this year. Her post-operative course was complicated by portal vein thrombosis (PVT), which may have been related to the trauma and operation rather than the ET. She is now noted to have a worsening thrombocytosis. Her previous physician started her on hydroxyurea, which she continues at this time. Her most recent complete blood count showed a white blood cell (WBC) count of 6.7 x109/L, a hemoglobin level of 12.4 gm/dL, mean corpuscular volume of 94.8 fL and a platelet count of 1,246 per mcL. Marrow showed typical features of ET without an increase in fibrosis, and storage iron was present. She also has a history of iron deficiency anemia following childbirth last year and is now on anticoagulation for the PVT. She seems quite young to be committed to hydroxyurea to control the marked thrombocytosis. I considered pegylated interferon, although she has ongoing mental health issues. What would you suggest with respect to cytoreductive therapy in this setting? How long would you continue the anticoagulation?
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