Abstract

Objective: Toevaluate the clinical approach, the diagnostic method and the most appropriate therapeutic management of thrombotic microangiopathies (TMA) in pregnancy, still leading killers in the obstetric area today.

Materials and methods: A large review of the international literature and available clinical studies has been carried out in order to define the current state of the art regarding TMA in pregnancy. In the light of this, 9 clinical cases, among 152 TMA cases, of pregnant women hospitalized and who gave birth in the Pisa University Hospital O.O. U.U. Gynecology and Obstetrics 1 and 2 from 2010 to 2019, were identified, analyzed and re-discussed.

Results: Analyzing the diagnostic method and the medical records, we made a critical review of these 9 cases, accurately analyzing the diagnoses made. Among these cases, 6 Thrombotic Thrombocytopenic Purpura (TTP), 2 HELLP Syndrome and 1 Atypical Hemolytic Uremic Syndrome (aHUS) were diagnosed during pregnancy. By analyzing the medical records, the diagnostic method and thetherapeuticmanagement of these patients, we questioned the diagnoses made. These diagnoses, from our analytical point of view, are partially not corresponding, being 4 cases of TTP and 5 possible cases of aHUS.

Conclusion: From the review of our case history, in the Pisa Obstetric clinics, it is possible to find an under diagnosis of the aHUS cases compared to those of TTP and HELLP syndrome, due both to the unavailability of the ADAMTS13 functionality test and to the unused LDH/AST ratio, which in our opinion could represent a future resource in diagnostic approach to thrombotic microangiopathies in pregnancy. 

Keywords

Thrombotic microangiopathies, Pregnancy, HELLP syndrome, Thrombotic thrombocytopenic purpura, Atypical hemolytic-uremic syndrome, Disseminated intravasal coagulation, Thrombocytopenia, Hemolytic anemia, Willebrand factor, Malignant hypertension, Bone marrow, Systemic lupus erythematosus, Tumors