Development of atypical hemolytic uremic syndrome after gynecologic surgery: clinical presentations, diagnosis, and treatment

Kirsanova T.V., Vinogradova M.A., Shmakov R.G.

Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia, Moscow 117997, Ac. Oparina str. 4, Russia
Objective. To carry out a systematic analysis of the data available in present-day literature on the types of thrombotic microangiopathy, the trigger for which may become gynecologic surgery.
Material and methods. The paper describes an extremely rare case of atypical hemolytic uremic syndrome (aHUS) in gynecological practice, which has an unusual clinical picture including deep vein thrombosis with pulmonary embolism (PE) in addition to acute renal failure.
Results. The paper depicts a differential diagnostic search between different types of thrombotic microangiopathy, which could be induced by gynecologic surgery. It considers all the triggers or complement-activating conditions, which result in aHUS, identifies the stages of diagnosis, and views the complexity of treatment, the choice of therapeutic approaches, and a quick response to targeted therapy with eculizumab.
Conclusion. The given clinical case showed a manifestation of aHUS with deep vein thrombosis and PE after diagnostic uterine curettage in the patient. Pathogenetic treatment initiated on day 7 after the onset of aHUS could rapidly achieve not only a hematologic response, but also restore the function of the kidneys and other affected organs.

Keywords

thrombotic microangiopathy
atypical hemolytic uremic syndrome
deep vein thrombosis
catastrophic antiphospholipid syndrome

Supplementary Materials

  1. Fig. 1. Dynamics of laboratory parameters of patient A.
  2. Fig. 2. Arterio-venous shunt at the level of the interlobar arteries, impoverishment of the cortical blood flow according to the ultrasound dopplerography data of the renal arteries

References

1. Bravo J.J., Novoa D., Romero R., Sanchez-Guisande D. Sindrome hemolitico uremico postmiomectomia. Nefrologia. 2001; 21(2): 217.

2. Taylor A., Sharma M., Buck L., Mastrogamvrakis G., DiSpezio Sardo A., Magos A. The use of triple tourniquets for laparoscopic myomectomy. J. Gynecol. Surg. 2005; 21: 65-72.

3. Zheng X.L., Sadler J.E. Pathogenesis of thrombotic microangiopathies. Annu. Rev. Pathol. Mech. Dis. 2008; 3: 249-77.

4. Kumar K.V., Jensen C.E., Singer A., Wonke B., Morgan H. A case of myomectomy complicated by disseminated intravascular coagulation. J. Obstet. Gynaecol. 1997; 17(3): 307-8.

5. Besbas N., Karpman D., Landau D., Loirat C., Proesmans W., Remuzz G. et al. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, and related disorders. Kidney Int. 2006; 70(3): 423-31.

6. Noris M., Remuzzi G. Atypical hemolytic-uremic syndrome. N. Engl. J. Med. 2009; 361(17): 1676-87.

7. Козловская Н.Л., Коротчаева Ю.В., Боброва Л.А., Шилов Е.М. Акушерский атипичный гемолитико-уремический синдром: первый российский опыт диагностики и лечения. Нефрология. 2016; 20(2): 68-80. [Kozlovskaya N.L., Korotchaeva Yu.V., Bobrova L.A., Shilov E.M. Obstetric atypical hemolytic-uremic syndrome: the first Russian experience in diagnosis and treatment. Nefrologiya. 2016; 20(2): 68-80. (in Russian)]

8. Tsimpanakos I., Connolly J., Alatzoglou K.S. Two cases of myomectomy complicated by intravascular hemolysis and renal failure: disseminated intravascular coagulation or hemolytic uremic syndrome? Fertil. Steril. 2010; 93(6): 2075. e11-2075. e15.

9. Sizzi O., Rossetti A., Malzoni M. Italian multicenter study on complications of laparoscopic myomectomy. J. Minim. Invasive Gynecol. 2007; 14(4): 453-62.

10. Swisher K.K., Doan J.T., Vesely S.K., Kwaan H.C., Kim B., Lämmle B. et al. Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports. Haematologica. 2007; 92(7): 936-43.

11. Kitchens C.S. Thrombotic storm: when thrombosis begets thrombosis. Am. J. Med. 1998; 104(4): 381-5.

12. Cervera R., Bucciarelli S., Plasin M.A., Gómez-Puerta J.A., Plaza J., Pons-Estel G. et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the «CAPS Registry». J. Autoimmun. 2009; 32(3-4): 240-5.

13. Krieg S., Studt J.D., Sulzer I., Lämmle B., Kremer Hovinga J.A. Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS 13 deficiency? Thromb. Haemost. 2005; 94(6): 1186-9.

14. Levey S.A., Bajwa R.S., Picken M.M., Clark J.I., Barton K., Leehey D.J. Thrombotic microangiopathy associated with sunitinib, a VEGF inhibitor, in a patient with factor V Leiden mutation. NDT Plus. 2008; 1(3): 154-6.

15. Ольхова Е.Б. Ультразвуковое исследование почек при гемолитико-уремическом синдроме у детей. Нефрология и диализ. 2001; 3(1): 61-6. [Olkhova E.B. Ultrasonography of kidneys with hemolytic-uremic syndrome in children. Nefrologiya i dializ. 2001; 3 (1): 61-6. (in Russian)]

16. Кирсанова Т.В., Козловская Н.Л., Кушнир В.В., Шахнова Е.А., Платова Е.Н., Садовников В.И., Беляева Л.Е. Ультразвуковая допплерография почечных сосудов в диагностике поражения почек при тромботических микроангиопатиях. Нефрология и диализ. 2008; 10(3-4): 219-25. [Kirsanova T.V., Kozlovskaya N.L., Kushnir V.V., Shakhnova E.A., Platova E.N., Sadovnikov V.I., Belyaeva L.E. Ultrasonic dopplerography of renal vessels in the diagnosis of kidney damage in thrombotic microangiopathies. Nefrologiya i dializ. 2008; 10 (3-4): 219-25. (in Russian)]

17. Reising A., Hafer C., Hiss M., Kielstein J.T., Menne J., Gueler F. et al Ultrasound findings in EHEC-associated hemolytic-uremic syndrome and their clinical relevance. Int. Urol. Nephrol. 2016; 48(4): 561-70.

18. Козловская Н.Л., Прокопенко Е.И., Эмирова Х.М., Серикова С.Ю. Клинические рекомендации по диагностике и лечению атипичного гемолитико-уремического синдрома. Нефрология и диализ. 2015; 17(3): 242-64. [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. 2015; 17(3): 242-64. (in Russian)]

Received 09.03.2017

Accepted 28.04.2017

About the Authors

Kirsanova T.V., PhD, Senior Researcher of the Therapeutic Department, Research Center of Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia.
117997, Russia, Moscow, Ac. Oparina str. 4
Vinogradova M.A., PhD, Head of the Division of Hematology and Clinical Reproductive Hemostasis, Research Center of Obstetrics, Gynecology and Perinatology,
Ministry of Health of Russia. 117997, Russia, Moscow, Ac. Oparina str. 4
Shmakov R.G., Doctor of Medicine, Head Physician, Research Center of Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia.
117997, Russia, Moscow, Ac. Oparina str. 4. Tel.: +74954381183. Е-mail: r_shmakov@oparina4.ru

For citations: Kirsanova T.V., Vinogradova M.A., Shmakov R.G. Development of atypical hemolytic uremic syndrome after gynecologic surgery: clinical presentations, diagnosis, and treatment. Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2018; (1): 154-60. (in Russian)
https://dx.doi.org/10.18565/aig.2018.1.154-160

Similar Articles

By continuing to use our site, you consent to the processing of cookies that ensure the proper functioning of the site.