AJA Asian Journal of Anesthesiology

Advancing, Capability, Improving lives

Correspondence
Volume 59, Issue 2, Pages 79-80
Yuma Kadoya 1 , Tomoyo Nishida 2 , Satoki Inoue 1 , Masahiko Kawaguchi 1
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Outline


To the Editor,

Thrombocytopenia is common in pregnancy and occurs in 5–10% of all cases.1 We report a case of postpartum thrombocytopenia that emulates thrombotic thrombocytopenic purpura (TTP). The tentative diagnosis of TTP prevented us from choosing an appropriate therapeutic option.

A 40-year-old woman was admitted to our intensive care unit (ICU) after an emergency cesarean section owing to preeclampsia. She developed pulmonary hypertension (PH) during pregnancy. Thrombocytopenia (reductions from 93,000 to 51,000 per mm3) in conjunction with the presence of schistocytes and the progression of PH was also observed on the 4th postoperative day (POD). There were no abnormal findings on other laboratory data including liver enzymes, contrast-enhanced computed tomography, and lung perfusion scintigraphy. Therefore, she was admitted to ICU for plasma exchange (PE) with the tentative diagnosis of TTP on the 6th POD. When a hemodialysis catheter was inserted, she developed cardiac arrest. Veno-arterial extracorporeal membrane oxygenation was used to maintain her life. Consecutive PEs were performed; however, no improvement was observed regarding thrombocytopenia. By contrast, massive bleeding occurred because platelet transfusion was a contraindication of TTP treatment. Subsequently, laboratory results dismissed the presence of TTP. Eculizumab was also administered because atypical hemolytic uremic syndrome (aHUS) was suspected. All of the treatments failed, and no improvements were apparent for both thrombocytopenia and PH. On the 16th ICU day, she died owing to multiple organ failure. Postmortem laboratory results denied aHUS.

The mortality rate of pulmonary arterial hypertension (PAH) among pregnant women is still very high (16%).2 Additionally, our case had no treatment for PH before ICU admission even though the risk of mortality increases during the first 4 weeks after delivery.3 Therefore, this case may have already been diffi cult to rescue at the time of ICU admission.

Additionally, the deprivation of her chance for survival may be attributed to the tentative diagnosis of TTP. HELLP syndrome was excluded due to the lack of elevated liver enzymes. The first-line therapy for TTP is PE, which requires a hemodialysis catheter. She developed cardiac arrest because of inadequate pain management when the hemodialysis catheter was inserted. Cardiac arrest might not have occurred without the tentative diagnosis of TTP. In addition, much earlier platelet transfusion, which has been recognized as a contraindication of TTP treatment, might have controlled massive bleeding adequately.

It was reported that TTP and aHUS are often mistaken for preeclampsia owing to the overlapping clinical and laboratory features and that delays in appropriate diagnosis and treatment may be life-threatening.4 In this case, TTP and aHUS were diagnosed only with thrombocytopenia and schistocytes, but this was dismissed based on the results of ADAMTS13 activity and the anti-complement factor H autoantibodies. A previous report shows that the most frequent abnormality in patients with pregnancy-related hypertensive disorders was the presence of schistocytes in up to 39% of samples.5 Therefore, the presence of schistocytes in our case was not attributed to TTP or aHUS. Besides, during the first several weeks after delivery, microthromboembolism of the lung arteries attributed to hypercoagulation can also occur.3 This may have contributed to the evident thrombocytopenia and deterioration of PH. Consequently, we diagnosed this case as deteriorated PAH because of no indications for other diseases.

Prior to the suspicion of TTP or aHUS based only on the presence of thrombocytopenia and schistocytes, we should have considered that these findings were the signs of deterioration of PAH during the postpartum period. Early implementation of the standard treatments for PAH and anticoagulation therapy might have changed her postpartum course.


References

1
Cines DB, Levine LD.
Thrombocytopenia in pregnancy.
Blood. 2017;130(21):2271-2277.
2
Meng ML, Landau R, Viktorsdottir O, et al.
Pulmonary hypertension in pregnancy: a report of 49 cases at four tertiary North American sites.
Obstet Gynecol. 2017;129(3):511-520.
3
Bédard E, Dimopoulos K, Gatzoulis MA.
Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?
Eur Heart J. 2009;30(3):256-265.
4
Gupta M, Feinberg BB, Burwick RM.
Thrombotic microangiopathies of pregnancy: differential diagnosis.
Pregnancy Hypertens. 2018;12:29-34.
5
Hernández Hernández JD, Villaseñor OR, Del Rio Alvarado J, et al.
Morphological changes of red blood cells in peripheral blood smear of patients with pregnancy-related hypertensive disorders.
Arch Med Res. 2015;46(6):479- 483.

References

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