Author Affiliations1Kemerovo Regional Clinical Hospital n.a, S.V. Belyaev 650066, Kemerovo, Oktyabr’sky av., 22; Kemerovo State Medical University of Minzdrav of Russia 650056, Kemerovo, Voroshilov str., 22a
2Kemerovo Regional Clinical Hospital n.a, S.V. Belyaev 650066, Kemerovo, Oktyabr’sky av., 22; Kemerovo State Medical University of Minzdrav of Russia 650056, Kemerovo, Voroshilov str., 22a
3Kemerovo Regional Clinical Hospital n.a, S.V. Belyaev 650066, Kemerovo, Oktyabr’sky av., 22
4Kemerovo Regional Clinical Hospital n.a, S.V. Belyaev 650066, Kemerovo, Oktyabr’sky av., 22; Kemerovo State Medical University of Minzdrav of Russia 650056, Kemerovo, Voroshilov str., 22a
5Kemerovo State Medical University of Minzdrav of Russia 650056, Kemerovo, Voroshilov str., 22a
6Kemerovo State Medical University of Minzdrav of Russia 650056, Kemerovo, Voroshilov str., 22a
The paper presents the case of clinical observation of a patient with atypical hemolytic-uremic syndrome (aHUS). aHUS is a disease characterized by an unfavorable prognosis (severe or catastrophic course with rapid development of terminal renal or multi-organ failure). The aim of the study is to evaluate the approaches to differential diagnosis of aHUS in clinical practice. Material and methods. The study was conducted on the basis of the Nephrology Department of Kemerovo Regional Clinical Hospital n.a. S.V. Belyaev. The clinical observation of patient D., aged 26 years old, is discussed. Results and discussion. Diagnosing aHUS requires: 1) diagnosing thrombotic microangiopathy (TMA: thrombocytopenia or decrease in platelet count by more than 25 % of original, visceral damage (kidneys, CNS, gastrointestinal tract, heart, lungs)); 2) ruling out HUS associated with Shiga toxin-producing Escherichia coli (STEC-HUS; negative for Shiga-toxin in blood and stool), thrombotic thrombocytopenic purpura (TTP), systemic connective tissue disease, catastrophic antiphospholipid syndrome, HIV infection; 3) assessing the activity of ADAMTS13 (decrease confirms the aHUS diagnosis); 4) proving normal content of complement components C3 and C4 as an additional argument in favor of aHUS diagnosis. At the first stage, the patient was diagnosed with TMA (platelet content 37 × 109/l, hemoglobin content 59 g/l), LDH up to 824 E/l), liver damage (AST, ALT and LDH activity 55, 60 and 824 U/l, respectively), kidney damage (acute renal damage), lungs, heart, and brain damage. At the second stage the following diagnoses were ruled out: STEC-HUS (Shiga toxin in blood and stool was not detected), TTP (ADAMTS13 activity level was 66 %, whereas reference values are 93–113 %, in TTP – below 5–10 %); systemic connective tissue diseases catastrophic antiphospholipid syndrome, HIV infection sepsis. Normal values of C3 (0.9 g/l) and C4 (0.23 g/l) complement components did not rule out the diagnosis of aHUS.
Afshar-Kharghan V. Atypical hemolytic uremic syndrome. Hematology Am SocHematolEduc Program. 2016; (1): 217-225. doi: 10.1182/asheducation-2016.1.217
Batyushin M. M., Rudenko L. I., Kozhin A. A., Golub V. V., Dan'kin O. N., Grekova I. A. Atypical hemolytic uremic syndrome. Clinical observations. Nefrologiya = Nephrology. 2014; 18 (5): 80-84. [In Russian].
Brocklebank V., Wood KM., Kavanagh D. Thrombotic microangiopathy and the kidney. Clin. J. Am. Soc. Nephrol. 2018; 13 (2): 300-317. doi: 10.2215/CJN.00620117.
Dem'yanova K.A., Kozlovskaya N.L., Bobrova L.A., Kozlov L.V., Andina S.S., Yurova V.A., Kuchieva A.M., Roshchupkina S.V., Shilov E.M. Complement system abnormalities in patients with atypical hemolytic uremic syndrome and catastrophic antiphospholipid syndrome. Vestnik Rossiyskoy akademii meditsinskikh nauk = Annals of the Russian academy of medical sciences. 2017; 72 (1): 42-52. [In Russian]. doi: 10.15690/vramn769
Jokiranta T.S. HUS and atypical HUS. Blood. 2017; 129 (21): 2847-2856. doi: 10.1182/blood-2016-11-709865
Kavanagh D., Raman S., Sheerin NS. Management of hemolytic uremic syndrome. F1000Prime Rep. 2014; 6: 119. doi: 10.12703/P6-119
Kozlovskaya N.L., Prokopenko E.I., Emirova Kh.M., Serikova S.Yu. Clinical recommendations for the diagnosis and treatment of atypical hemolytic uremic syndrome. Nefrologiya i dializ = Nephrology and Dialysis. 2015; 17 (3): 242-264. [In Russian].
Kobbe R., Schild R., Christner M., Oh J., Loos S., Kemper M. J. Case report - atypical hemolytic uremic syndrome triggered by influenza B. BMC Nephrol. 2017; 18 (1): 1-4. doi: 10.1186/s12882-017-0512-y
Loirat C., Frémeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J. Rare Dis. 2011; 6: 60. doi: 10.1186/1750-1172-6-60
Lora Sh., Fremyu-Bachi V. Atypical hemolytic uremic syndrome. Nefrologiya = Nephrology. 2012; 16 (2): 16-48. [In Russian]. doi: 10.1186/1750-1172-6-60
Rafiq A., Tariq H., Abbas N., Shenoy R. Atypical hemolytic-uremic syndrome: a case report and literature review. Am. J. Case Rep. 2015; 16: 109–114. doi: 10.12659/AJCR.892907
Rodriguez E., Barrios C., Soler M. J. Should eculizumab be discontinued in patients with atypical hemolytic uremic syndrome? Clin. Kidney J. 2017; 10 (3): 320–322. doi: 10.1093/ckj/sfx024
Yenerel M.N. Atypical hemolytic uremic syndrome: Differential diagnosis from TTP/HUS and management. Turk. J. Haematol. 2014; 31 (3): 216–225. doi: 10.4274/tjh.2013.0374
Yoshida Y., Kato H., Ikeda Y., Nangaku M. Pathogenesis of atypical hemolytic uremic syndrome. J. Atheroscler. Thromb. 2019; 26 (2): 99–110. doi: 10.5551/jat.RV17026
Zhang K., Lu Y., Harley K.T., Tran M.H. Atypical hemolytic uremic syndrome: a brief review. Hematol. Rep. 2017; 9 (2): 7053. doi: 10.4081/hr.2017.7053