Management of Early Pregnancy Failure in a Patient with Pancytopenia

C l i n M e d International Library Citation: Cholakian D, Coleman J (2014) Management of Early Pregnancy Failure in a Patient with Pancytopenia. Gynecol Cases Rev 1:018 Received: October 28, 2014: Accepted: December 04, 2014: Published: December 08, 2014 Copyright: © 2014 Cholakian D. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Cholakian and Coleman. Obstet Gynecol cases Rev 2014, 1:3 ISSN: 2377-9004


Introduction
Tnpolyagglutination Syndrome (TnP) is a rare hematologic disorder characterized by pancytopenia with multi-lineage dysplasia [1].In most cases of TnP, a defect in the synthesis of blood cell membrane glycoproteins (Gb3/CD77) allows exposure of the normally hidden Tn antigen to naturally occurring anti-Tn antibodies, thereby leading to polyagglutination [1][2][3][4].The defect in the synthesis of this membrane is thought to be due a single point mutation in the Gb3/CD77 synthase and can also be found on the membranes of platelets, granulocytes, and lymphocytes [1,5].Prevalence is unknown since it is estimated that many cases of Tnpolyagglutination are undiagnosed due to modern blood typing [2].Previously, pooled human adult sera, which contains large amounts of naturally occurring anti-Tn, was used for ABO typing and TnP was suspected if there was diffuse polyagglutination of red blood cells, regardless of the ABO type [4].Since monoclonal reagents are used for these tests today, cases of polyagglutination are not so obvious, and they usually are not noticed until human serum and red cells are mixed together in performance of an antibody screen or cross match.While TnP is not directly linked to malignancy, case reports of acute leukemia in patients with Tnpolyagglutination syndrome have been published [6].
Here we present the management of an embryonic demise in a woman with TnP.
8.7g/dL, platelets 67,000cells/microliter, normal coagulation profile, serum human chorionic gonadotropin 173.5mIU/mL, and Rh positive (Figure 1).A septic abortion was diagnosed, IV cefotetan was administered, and a suction dilation and curettage (D&C) was performed.Blood loss was 250 milliliters and unremarkable products of conception were obtained.
Twelve hours post-operatively, the patient spiked a temperature to 41°C and the antibiotic was changed to ertapenem.On post-operative day (POD)-1, pancytopenia worsened: WBC 0.61, ANC 240, hemoglobin 6.5, and platelets 40,000.Blood and urine cultures were negative as were gonorrhea/chlamydia tests.Hematology was consulted and a secondary consumptive process was ruled-out.Bone marrow biopsy was not performed because the severe neutropenia was attributed to acute infection.The patient declined packed red blood cell (PRBC) and platelet transfusions given minimal vaginal bleeding and lack of symptoms.On POD-4, she was afebrile without complaints or examination findings.Laboratory values improved (Figure 1).Ten days of amoxicillin/clavulanate, iron supplements, and NuvaRing were prescribed.Four weeks after hospitalization, she was doing well and laboratory values were back tobaseline.

Discussion
To our knowledge, this is the first report of the management of early pregnancy failure in a patient with pancytopenia caused by TnP.Management of early pregnancy failure in patients with pancytopenia requires careful evaluation, as there may be increased risk of infection and bleeding.
Risk of infection increases as the ANC decreases.Some experts recommend hospitalization and parenteral antibiotics if the ANC drops below 500 [7].Despite the seven hospitalizations for infections, this patient never required intensive care, and was aggressively managed with broad-spectrum antibiotics.At the time of the embryonic demise, ANC was greater than 1300, and there was moderate anemia and thrombocytopenia.Although expectant and medical management with misoprostol are not contra indicated, in retrospect, uterine aspiration might have been the best option.Initial surgical management might have avoided the additional ER visit, hospitalization, and IV antibiotics.
Second, none of her pregnancies resulted in hemorrhage or required transfusion of PRBCs despite anemia and thrombocytopenia.Post-operatively, patient's laboratory values dropped below transfusion thresholds for PRBCs (<7) and platelets (<50,000); however, she declined transfusion.If the patient had agreed, blood products need not be washed prior to transfusing because donor anti-Tn would be diluted with anti-coagulant and unlikely to cause hemolysis in vivo [8][9][10].
In sum, there is comparable risk of bleeding, infection, and curettage between medical and surgical management in healthy women, however these data are not available for patients with pancytopenia [11].Other counseling issues include amount of pain or bleeding to expect, number of unscheduled clinic visits, time to completion of abortion, risk of hospitalization, and surgical complications.This case demonstrates that uterine aspiration should be strongly considered as first line; additionally, cross-matched blood products should be available, and prophylactic post-operative broadspectrum antibiotics may be useful in women with severe neutropenia (ANC<500).Given that surgical management allows for monitoring of a high risk patient in a controlled, hospital setting, consideration should be made to offer it as first line in patients with pancytopenia.Overall, the decision making process is complex in women with TnP given the lack of available data to guide management.This case demonstrates that obstetric or gynecologic complications are managed fairly well in patients with TnP, if promptly evaluated and empiric parenteral therapy is implemented.