Retrospective Analysis of Patients Hospitalized with the Diagnosis of Microangiopathic Hemolytic Anemia in our Internal Medicine Clinic

Abstract

Introduction: Microangiopathic hemolytic anemia (MAHA) is a group of diseases rarely seen and having severe mortality rate if it doesn’t treated. Aim: Our aim is to emphasize the need to recall the MAHA in differential diagnosis of patients with thrombocytopenia, especially those without end-organ involvement, which are presented in this study. At the same time to show the rate of mortality decreases in early diagnosis and treatment. Materials and methods: We retrospectively reviewed the cases that were diagnosed and examined and treated in the Internal Medicine Service between september 2017 and may 2019. A total of 15 patients were included in the study. From patients, first application; complete blood count, sedimentation, peripheral smear, direct coombs, CRP, coagulation tests and biochemical tests were performed. In addition, before and after each plasmapheresis procedure; complete blood count, urea, creatinine, LDH, indirect bilirubin levels were measured.Findings: Gender ratio of the patients was 8 / 7 (53.3 / 46.6) and the mean age was 46.8 (25-79). As a result of etiological classification of patients; 8 patients (53.3%) had TTP (Thrombotic thrombocytopenic purpura), 4 patients (%26.6 ) had vitamin B12 deficiency, 1 patient had systemic lupus erythematosus ( %6.6), 1 patient (%6.6) was associated with complement, 1 patient (%6.6) had drug-releated MAHA. Conclusion : Of the 15 patients included in the study, 11 patients (73.3%) were recovered, 1 patient (6.6%) died after discharge, and 4 patients (20%) died during follow, totally 5 (%33.3) patients died.

Keywords

• Anemia
• hemolytic
• retrospective

İç Hastalıkları Servisimizde Mikroanjiopatik Hemolitik Anemi (MAHA) Tanısı İle Yatırılarak Takip Ettiğimiz Hastaların Retrospektif Analizi

Abstract

Giriş: Mikroanjiopatik hemolitik anemiler (MAHA) oldukça nadir görülen ve tedavi edilmediğinde mortal seyreden bir grup hastalığa verilen isimdir.
Amaç: Bu çalışmadaki amacımız; trombositopeni saptanan ve özellikle de end-organ tutulumu olmayan hastalarda ayırıcı tanıda MAHA’nın hatırlanması gerekliliğini vurgulamaktır. Aynı zamanda erken tanı ve tedavide mortalite oranının düştüğünü gösterebilmektir. Gereç ve yöntem: Eylül 2017- mayıs 2019 tarihlerinde arasında iç hastalıkları servisinde tanısı koyularak takip ve tedavi edilen vakaları retrospektif olarak inceledik. Tanı kriterlerine uyan toplam 15 hasta çalışmaya alındı. Hastalardan, ilk başvuruda; tam kan sayımı, sedimentasyon, periferik yayma, direkt coombs, CRP, koagülasyon testleri ve biyokimyasal tetkikler çalışıldı. Ayrıca her plazmaferez işleminden önce ve sonra; tam kan sayımı, üre, kreatin, LDH, indirek bilirubin düzeyleri bakıldı. Bulgular: Hastaların E/K oranı 8 /7 (% 53.3/ 46.6) ve yaş ortalaması 46.8 (25-79) bulundu. Hastaların etyolojik sınıflandırılması sonucu; 8 hasta (%53.3) TTP(Trombotik trombositopenik purpura) olarak, 4 hasta (%26.6) vitamin B 12 eksikliğine bağlı, 1 hasta sistemik lupus eritamatozus (%6.6), 1 hasta (%6.6) atipik hemolitik üremik sendrom ve 1 hasta (%6.6) da ilaç ilişkili MAHA olarak değerlendirildi. Sonuç: Çalışmaya alınan 15 hastadan 11 hastanın (%73.3) iyileşme, 4 hastanın (%20) izlemi esnasında exitus, 1 hastanın (%6.6) taburculuktan sonra, toplamda 5 (%33.3) hastanın exitus olduğu tespit edildi.

Keywords

• Anemi
• hemolitik
• retrospektif

References

1. 1-George JN, Nester CM. Syndromes of thrombotic microangiopathy. New Engl J Med 2014;371:654-666.
2. 2-Hoving J.A. Vesely S.K, Terrell DR. Et al. Survival and relapse in patient with thrombotic thrombocytopenic purpura. Blood 2010; 115:1500.
3. 3- Torok TJ, Holman RC& Chorba TJ. Increasing mortality from thrombotlc thrombocytopenic purpura in the United States: analysis of national mortality data. Am J Hematol 1995; 50: 84-90.
4. 4- Sadler JE, Moake JL, Miyata T, et al. Recent advances in thrombotic thrombocytopenic purpura. Hematology 2004; 407-23.
5. 5- Palermo MS, Exeni RA, Fernández GC. Hemolytic uremic syndrome: pathogenesis and update of interventions. Expert Rev Anti Infect Ther 2009;7(6):697-707.
6. 6- Dlott JS, Danielson CF, Blue-Hnidy DE, McCarthy LJ. Druginduced thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a concise review. Ther Apher Dial 2004;8(2):102-11 7- Shatzel JJ, Taylor JA. Syndromes of Thrombotic Microangiopathy. Med Clin North Am 2017;101:395-415.
7. 8- George JN. Cobalamin C deficiency-associated thrombotic microangiopathy: uncommon or unrecognised? Lancet 2015;386:1012.
8. 9- Scully M, Cataland S, Coppo P, de la Rubia J, Friedman KD, Kremer Hovinga J, Lämmle B, Matsumoto M, Pavenski K, Sadler E, Sarode R, Wu H; International Working Group for Thrombotic Thrombocytopenic Purpura. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost 2017;15:312-322.

9. 10- Song D, Wu LH, Wang FM, Yang XW, Zhu D, Chen M, Yu F, Liu G, Zhao MH. The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther 2013;15:R12.
10. 11- Mauro M, Kim J, Costello C, Laurence J. Role of transforming growth factor betal inmicrovascular endothelial cell apoptosis associated with thrombotic thrombocytopenic purpuraand hemolytic uremic syndrome. Am J Hemato 2001; 66(1): 12-22.
11. 12- KV.Chow .Anti-CD20 antibody in thrombotic thrombocytopenic purpura refractory to plasma exchange. Internal medicine journal (Intern Med J) 2007; 37:329-32