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Annals of Hematology

, Volume 96, Issue 10, pp 1715–1726 | Cite as

Clinical dissection of thrombotic microangiopathy

  • Eunjeong Kang
  • Shin Hye Yoo
  • Doyeun Oh
  • Kwon Wook Joo
  • Yon Su Kim
  • Sung-Soo Yoon
  • Inho Kim
  • Seonyang Park
  • Hajeong LeeEmail author
  • Youngil KohEmail author
Original Article

Abstract

Differential treatment strategies are applied in thrombotic microangiopathy (TMA) according to the sub-classifications. Hence, it is worthwhile to overview clinical manifestations and outcomes of overall TMA patients according to sub-classifications. We analyzed TMA patients whose serum lactate dehydrogenase levels >250 IU/L, with the presence of schistocytes in their peripheral blood smear, or with typical vascular pathologic abnormalities in their renal biopsy. We compared clinical manifestations including overall survival (OS) and renal survival according to TMA causes. A total of 117 TMA patients (57 primary and 60 secondary TMA) were analyzed. Renal symptom was the most common manifestation in whole patients, while renal function at diagnosis was worst in pregnancy-related TMA group. Primary TMA patients had more frequent CNS symptom and hematologic manifestation compared to secondary TMAs. Among secondary TMAs, pregnancy- and HSCT-related TMA patients showed prevalent hemolytic features. During 150.2 months of follow-up, 5-year OS rate was 64.8%. Poor prognostic factors included older age, combined hematologic and solid organ malignancies, lower hemoglobin levels, and lower serum albumin levels. There was no significant difference in OS between primary and secondary TMAs. Seventy-eight percent of patients experienced AKI during TMA. Five-year death-censored renal survival rate was poor with only 69.2%. However, excellent renal outcome was observed in pregnancy-associated TMA. TMA showed various clinical manifestations according to their etiology. Notably, both OS and renal survival were poor regardless of their etiologies except pregnancy-associated TMA. Physicians should differentiate a variety of TMA categories and properly manage this complex disease entity.

Keywords

Thrombotic microangiopathy Clinical manifestation Hemolytic uremic syndrome 

Notes

Compliance with ethical standards

This study was approved by the institutional review board at our institution (number: H-1509-070-703). All procedures were performed in accordance with the principles of the Declaration of Helsinki.

Conflict of interest

The authors declare that they have no conflict of interest.

Research involving human participants

For this type of study, formal consent is not required.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

277_2017_3063_MOESM1_ESM.docx (16 kb)
Supplementary Table 1 (DOCX 16 kb).

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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  • Eunjeong Kang
    • 1
  • Shin Hye Yoo
    • 1
  • Doyeun Oh
    • 2
  • Kwon Wook Joo
    • 3
    • 4
  • Yon Su Kim
    • 3
    • 4
  • Sung-Soo Yoon
    • 5
  • Inho Kim
    • 5
  • Seonyang Park
    • 5
  • Hajeong Lee
    • 3
    • 4
    Email author
  • Youngil Koh
    • 5
    Email author
  1. 1.Department of Internal MedicineSeoul National University College of MedicineSeoulSouth Korea
  2. 2.Division of Hematology-oncology, Department of Internal MedicineCHA University School of MedicineSeongnamSouth Korea
  3. 3.Division of Nephrology, Department of Internal MedicineSeoul National University College of MedicineSeoulSouth Korea
  4. 4.Kidney Research InstituteSeoul National University College of MedicineSeoulSouth Korea
  5. 5.Division of Hematology, Department of Internal MedicineSeoul National University College of MedicineSeoulSouth Korea

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