Splenectomy for HIV-Related Immune Thrombocytopenia Comparison with Results of Splenectomy for Non-HIV Immune Thrombocytopenic Purpura
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ORIGINAL ARTICLE Splenectomy for HIV-Related Immune Thrombocytopenia Comparison With Results of Splenectomy for Non-HIV Immune Thrombocytopenic Purpura Reginald V. N. Lord, FRACS; Maxwell J. Coleman, FRACS; Samuel T. Milliken, FRACP Objective: To determine the effectiveness and safety of tomy, with an elevation of the platelet count to greater splenectomy for patients with human immunodefi- than 1003109/L. After a median follow-up of 26.5 months, ciency virus (HIV)–related immune thrombocytopenia, all but 1 patient had a sustained complete remission with using the results of splenectomy for patients with non- no need for medical therapy for thrombocytopenia. Sple- HIV immune thrombocytopenic purpura as a control nectomy was more effective in the HIV-related throm- group for comparison. bocytopenia group than in the non-HIV immune throm- bocytopenic purpura group, with significantly higher Design: Retrospective study. platelet counts at 1 week and 1 month after splenec- tomy in the HIV group (t test, P=.02 and P=.009, respec- Setting: Tertiary care university hospital. tively). There were significantly fewer patients needing medical therapy for thrombocytopenia after splenec- Patients: Fourteen patients who underwent splenec- tomy in the HIV group (x2 test, P=.02). There were no tomy for symptomatic, medically refractory HIV- remarkable short- or long-term complications in the pa- related immune thrombocytopenia at this hospital from tients with HIV infection, including no overwhelming 1988 to 1997. During the same period, 20 patients had postsplenectomy infections. Three patients have died, and splenectomy for treatment of non-HIV immune throm- 2 patients have developed AIDS since operation. bocytopenic purpura. Conclusions: Splenectomy is effective treatment for pa- Intervention: Splenectomy. tients with symptomatic HIV-related thrombocytopenia that is resistant to medical therapy. The effectiveness of Main Outcome Measures: Platelet response, need for this treatment suggests that the predominant mecha- postsplenectomy medical therapy, progression of HIV dis- nism of thrombocytopenia in HIV-infected patients is in- ease, and complications. creased destruction of platelets because of platelet- associated immunoproteins. Results: All patients with HIV-related thrombocytope- nia had a complete early platelet response to splenec- Arch Surg. 1998;133:205-210 SYNDROME of immune tients having a platelet count less than thrombocytopenic pur- 1503109/L.3-6 In one large study of HIV- pura (ITP) in homo- infected patients, 5.3% of patients had se- sexual men, now recog- vere thrombocytopenia, with a platelet count nized to be due to human less than 503109/L.3 HIV-related thrombo- Aimmunodeficiency virus (HIV) infec- cytopenia may develop at any stage of HIV tion, was first reported in 1982 by Morris infection; it is not an acquired immunode- et al.1 Similar in many respects to the dis- ficiency syndrome (AIDS)–defining ill- ease termed either idiopathic or immune ness, and it occurs in HIV-infected pa- thrombocytopenic purpura in the non- tients from all HIV high-risk groups. HIV general population, this syndrome has Although one study reported a higher been variously named HIV-related throm- prevalence of HIV-related thrombocyto- bocytopenia, HIV-related (immune) penia in patients with AIDS than in thrombocytopenia, and HIV-associated HIV-infected patients without AIDS,4 the From the Departments of 2 Surgery (Drs Lord and thrombocytopenia. development of thrombocytopenia is gen- Coleman) and Hematology Thrombocytopenia is a common erally thought not to indicate worsened im- (Dr Milliken), St Vincent’s complication of HIV infection, with be- munodeficiency, and patients with HIV- Hospital, Sydney, Australia. tween 10% and 20% of HIV-infected pa- related thrombocytopenia do not get AIDS ARCH SURG/ VOL 133, FEB 1998 205 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 SUBJECTS AND METHODS For patients who had splenectomy for HIV-related thrombocytopenia, the following additional information was recorded: year of HIV diagnosis, month and year of The medical records of all patients who had splenectomy HIV-related thrombocytopenia diagnosis, risk factors for at St Vincent’s Hospital between January 1, 1988, and Janu- HIV infection, and stage of HIV disease at the time of ary 1, 1997, were retrospectively reviewed. Before 1988, operation. Staging was according to the Centers for Dis- HIV infectivity was not included on the computer-based ease Control and Prevention (CDC) 1993 revised classifi- record system of admissions to this hospital. Permission cation,31 in which group 1 is acute HIV syndrome (sero- to review and report on the medical files of HIV-infected conversion illness), group 2 is asymptomatic infection, patients was obtained from the New South Wales Depart- and group 3 is progressive generalized lymphadenopathy. ment of Health and the National Centre of HIV Epidemi- Group 4 is AIDS, defined by the CDC case definitions for ology and Clinical Research. Follow-up information was AIDS, and including in the revised classification all HIV- obtained from the medical records, by postal and tele- infected persons who have less than 0.203109/L CD4+ T phone inquiry with the patients’ local physicians or their lymphocytes (200/µL), or a CD4+ T lymphocyte percent- hematologists, and with the patients. Follow-up informa- age of total lymphocytes of less than 14. The CD4+ count tion was available for all patients. Patients were included measured closest to operation was recorded for each in the non-HIV ITP group if they had been diagnosed as patient, provided that the cell count was measured within having ITP and had isolated thrombocytopenia with no clini- 3 months of operation. cally apparent associated conditions or other causes of Symptoms and signs of thrombocytopenia were re- thrombocytopenia.9 corded for the HIV-related thrombocytopenia group, as were The following information was recorded for patients the details of medical treatment for thrombocytopenia. Only who had splenectomy as treatment for either HIV-related medications given for thrombocytopenia more than 3 thrombocytopenia or non-HIV ITP: age, sex, diagnosis, date months after splenectomy were recorded, to avoid record- of splenectomy, intraoperative and postoperative compli- ing the use of drugs that were being gradually weaned af- cations, and length of postoperative stay. All splenecto- ter operation. Also noted were the results of histopatho- mies were performed through a midline incision. The low- logical examination of the spleen, and whether vaccines for est platelet count before splenectomy, the platelet count 1 encapsulated organisms were given. Follow-up informa- week after splenectomy, and any other available platelet tion recorded included whether there had been progres- counts, were also recorded. End points for recording of post- sion of HIV disease, and whether any severe or overwhelm- operative platelet counts were either the last available count ing infections had occurred. or the last count before institution of medical therapy for Variables for the 2 groups who underwent splenec- thrombocytopenia for patients who relapsed or remained tomy for HIV-related thrombocytopenia or for non-HIV ITP severely thrombocytopenic after splenectomy. were compared using the Student t test and the x2 test. sooner than others.3 Spontaneous remission may occur karyocytes are seen in the bone marrow in both condi- in up to 20% of cases,5,7 and splenectomy is only re- tions, and the spleen is typically of normal size in both quired for the minority of patients who have severe, symp- conditions.19 tomatic, medically resistant thrombocytopenia. There are differences between the 2 diseases, how- There are many similarities between classic ITP in ever. Classic ITP, unlike HIV disease in this society, more non-HIV infected individuals and HIV-related thrombo- commonly affects women, and spontaneous remission cytopenia. Clinical features for both conditions, if pres- may be more common in HIV-related thrombocytope- ent, are the same. These include spontaneous or easy nia. Antiretroviral nucleoside analog drugs such as zi- bruising or bleeding, petechiae, gastrointestinal tract hem- dovudine (AZT) may be helpful in the treatment of HIV- orrhage, and (uncommonly) intracranial hemorrhage. A related thrombocytopenia but are not used for patients rise in the platelet count after administration of cortico- with non-HIV ITP. Unlike classic primary ITP, but simi- steroids and intravenous immunoglobulin is usually ob- lar to secondary ITP that follows a viral infection or drug served in both conditions, although this rise is often not reaction, immune complexes may be more important than sustained after withdrawal of the drugs. Splenectomy is antiplatelet antibodies in the pathophysiology of HIV- effective long-term therapy for patients with classic related thrombocytopenia.20,21 There is also evidence that chronic ITP,8,9 and despite concerns that splenectomy in defective production of platelets, rather than just in- HIV-infected patients might increase the risk of devel- creased destruction of platelets as in ITP, is occurring in oping AIDS10 or overwhelming postsplenectomy sepsis, at least some cases of HIV-related thrombocytope- splenectomy has been successfully used for the treat- nia.22-26 The production defect is at the megakaryocyte ment of HIV-related thrombocytopenia.2,11-17 level, perhaps due to infection of the