Benign chronic immune thrombocytopenic purpura in adult males
Authors: Beverly W. Baron, Joseph M. Baron
Journal: American Journal of Hematology (2016)

To the Editor: The definition of immune thrombocytopenic purpura (ITP) is isolated thrombocytopenia without clinically apparent associated conditions or other causes of thrombocytopenia. No specific criteria establish this condition; rather, the diagnosis relies on the exclusion of alternative disorders 1. Non-immune causes of thrombocytopenia, e.g., exposure to drugs or toxins, splenic platelet sequestration, primary bone marrow disorders, radiation exposure, and inherited thrombocytopenias 2 must be excluded. Diverse clinical features and responses to treatment suggest that this disorder may derive from heterogeneous mechanisms 2. ITP evolves into a chronic disease (thrombocytopenia for more than twelve months) in 80% of adults 2 and may be resistant to conventional therapy. Bleeding symptoms, which are the most common clinical manifestation of ITP, are more frequent in the older age group and may occur without a precipitating event. Intracranial hemorrhage is the most feared complication 2. Adults with ITP have increased morbidity and mortality as compared with the general population, especially those not able to maintain a platelet count above 30 × 109 despite treatment. The prognosis of ITP may become more serious with increasing age; however, there is considerable variability in the clinical course of adults with chronic ITP 3, 4. We have encountered several cases of mild to moderate chronic ITP in adult males in whom the disease was diagnosed incidentally and was stable over prolonged periods (6–20 years). No additional conditions developed, and no treatment was necessary. A review of their history and laboratory data was performed with their consent under an IRB-approved protocol. Although the number of cases we report is small, we believe it is important to recognize that this subset of patients, similar to those noted earlier 5, is a part of the spectrum of adults with chronic ITP. Case 1: A 51-year-old Caucasian male physician was noted to have a lowered platelet count of 136 × 103/µL in 1996. Other blood counts were within normal limits (Hb 14.1 g/dL, Hct 41.4%, WBC 7 × 103/µL). Since 2005 platelet counts ranged between 73 and 140 × 103/µL. Platelet autoantibody workup in 2009 showed the platelet eluate was positive for antibodies directed against platelet glycoproteins (GP) IIb/IIIa and 1b/IX, findings consistent with a diagnosis of ITP. Other medical conditions included subtotal thyroidectomy for goiter in 2003, atrial fibrillation, and dyslipidemia. The most recent platelet count is 100 × 103/µL. The patient has had no symptoms related to ITP, and he has not received any treatment for it. Case 2: A 47-year-old Caucasian male surgeon was found to have a platelet count of 123 × 103/µL in 2004. Other blood counts were within normal limits (Hb 16 g/dL, Hct 45.7%, WBC 5.3 × 103/µL). Platelet counts since then have ranged from 95 to 139 × 103/µL. Platelet autoantibody testing (2011) was positive for plasma antibodies directed against platelet GP Ib/IX. Direct antiglobulin testing (DAT) was negative (2011). Drug-dependent platelet antibody screen was negative for hydrochlorothiazide and losartan potassium. Other medical conditions have included obesity, nonalcoholic fatty liver, obstructive sleep apnea, adenomatous colonic polyps, and renal cyst. He has had no symptoms related to his thrombocytopenia, and he has had no therapy for it. Case 3: A 58-year-old Caucasian male mathematics teacher was first noted to have a reduced platelet count of 134 × 103/µL in 2010; WBC was said to be normal, Hb was 12.4 g/dL (patient was undergoing weekly phlebotomy for a diagnosis of hereditary hemochromatosis associated with a gene mutation). Platelet autoantibody testing (Aug. 2011) showed that the platelet eluate was positive for antibodies to GPIIb/IIIa and GPIa/IIa—consistent with a diagnosis of ITP. The DAT was negative. The most recent available platelet count was 100 × 103/µL. Other medical conditions include osteoarthritis (status post hip replacement) and hyperlipidemia. There have been no symptoms related to his thrombocytopenia, and he has not been treated for it. Case 4: We encountered a fourth case of a middle aged man with ITP similar to those above. However, the details of this case cannot be described here because the patient did not give his permission. ITP generally has been believed to be more common in women than in men, although one study has suggested that, in individuals over the age of 70, it is more common in males 2. Kueh 5 reported four male subjects over age 55 similar to ours with incidentally noted mild to moderate thrombocytopenia who had a benign course. They were followed for at least five years. Although often benign in young individuals, ITP frequently is associated in older subjects with other conditions, such as lymphoid malignancies (e.g., lymphoma, chronic lymphocytic leukemia), connective tissue diseases, and infections (e.g., Helicobacter pylori, HIV, hepatitis C) 6. We have reported three men ranging in age from 47 to 58 years when diagnosed with ITP in whom the disease was stable over a six to 20-year follow-up. No other associated conditions developed, and no treatment was needed. However, these patients do need to be followed closely in the event that they develop viral infections or need medications which may precipitate a decrease in platelet count with attendant bleeding risk. Our patients confirm that the clinical course of chronic ITP can be quite variable and may be benign in a subset of middle-aged males who do not necessarily develop progressive platelet decline or an associated condition such as lymphoid malignancy, connective tissue disease, or chronic infection. Beverly W. Baron1* and Joseph M. Baron2 1Department of Pathology, University of Chicago, Chicago, Illinois; 2Department of Medicine, University of Chicago, Chicago, Illinois