Sequential thrombosis and bleeding in a woman with a prolonged activated partial thromboplastin time

Spencer, Akpan; Pearce, Michael I.

doi:10.1186/1477-9560-9-16

Cited by 6 publications

(11 citation statements)

References 30 publications

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“…The mode of presentation remains similar even with the variation in the days of occurrence. Our patient reported the occurrence of a massive hemorrhagic disease on the very first day post-LSCS, while Spencer et al [ 9 ] have reported the occurrence of massive hematomas in a primigravida five weeks post-LSCS. In another study, Lee et al [ 8 ] have reported a vaginal bleed five days after the LSCS, and the development of large hematomas 65 days after the LSCS.…”

Section: Discussionmentioning

confidence: 53%

Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report

Azam

Batool

Malik

et al. 2020

Cureus

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Acquired hemophilia A (AHA) is a bleeding diathesis caused by the production of autoantibodies to factor VIII (FVIII). It manifests as an isolated deranged activated partial thromboplastin time (aPTT) indicating a defect in the intrinsic coagulation pathway. Herein, we report a case of a 26-year-old woman who presented with hemoperitoneum in the postpartum period following a lower segment Caesarean section (LSCS). AHA carries significant mortality if it remains undiagnosed, and early recognition and measures to eradicate the acquired inhibitors are the mainstays of its management.

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Section: Discussionmentioning

confidence: 53%

Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report

Azam

Batool

Malik

et al. 2020

Cureus

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“…We identified 15 cases of an LA coexisting with AHA (n ¼ 14) and AHB (n ¼ 1) where the laboratory presentation was a prolonged aPTT: the initial clinical presentation was vascular occlusion in 6 cases [6][7][8][9][10][11] and bleeding in 9 cases. 9,[12][13][14][15][16][17][18] The different tests to diagnose LA, FVIII levels, and its inhibitors are shown in Table 1.…”

Section: Resultsmentioning

confidence: 99%

“…Vessel occlusion was the clinical presentation in 6 of the 15 cases of which 5 were deep vein thrombosis (DVT) 6-10 and 1 was ischemic stroke. 11 Of the 5 DVT cases, 2 developed postpartum, 6,9 1 in the absence of an underlying disorder, 7 1 during the course of non-Hodgkin lymphoma, 8 and 1 during treatment for chronic lymphoid leukemia. 10 Ischemic stroke developed in 1 patient with AHB having underlying insulin-dependent diabetes mellitus.…”

Section: Coexistent La and Ah Presenting As Thrombosismentioning

confidence: 99%

“…11 These underlying conditions may have contributed to the development of thrombosis. This was accompanied by bleeding in 3 cases where the FVIII level was 2 IU or less, 6,7,9 whereas it was the only clinical manifestation in the patients with lymphoma where FVIII was 12 IU and FIX was 28 IU, 8 although the patients with chronic lymphatic leukemia developed a fatal pulmonary embolism despite an unmeasurable FVIII level. 10 Conversely, warfarin may have contributed to the development of the hematoma in the first patient listed in Table 1 6 who presented a week earlier with DVT.…”

Section: Coexistent La and Ah Presenting As Thrombosismentioning

confidence: 99%

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Prolonged Activated Partial Thromboplastin Time

Ames

Graf

Archer

et al. 2014

Clin Appl Thromb Hemost

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To review the diagnostic difficulties of a prolonged activated partial thromboplastin time (aPTT) when 2 inhibitors with opposite clinical presentations coexist, we searched MEDLINE from January 1970 to November 2013 using acquired, factor VIII (FVIII), factor IX, hemophilia A and B, inhibitor, lupus anticoagulant (LA), antiphospholipid, anticardiolipin, anti-β2-glycoprotein I, antibodies, syndrome, bleeding, and thrombosis. We identified 13 articles for a total of 15 cases of possible coexistence of FVIII inhibitor and LA. The presenting clinical manifestation was thrombosis in 6 cases and bleeding in 9 cases. Activated partial thromboplastin time was the presenting laboratory abnormality in all cases, and first-line investigations suggested the coexistence of LA and acquired FVIII inhibitor. None of the articles addressed the diagnostic accuracy of the screening tests by performing "second line" assays. We reviewed the diagnostic pitfalls of the cases under study and provide some guidance for alternative tests when facing a prolonged aPTT that may have a double meaning.

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“…We performed a literature review and identified nine cases of patients with acquired hemophilia A and concurrent thrombosis ( Table 1 ). 13 15 16 17 18 19 20 Most cases described the development of bleeding on the background of ongoing treatment for venous thrombosis. Several cases in the literature also discuss the difficulty of discriminating a DVT from an intramuscular hematoma or bleeding, with superimposed bruising and secondary extrinsic venous outflow obstruction.…”

Section: Discussionmentioning

confidence: 99%

Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies

Chhabra

Hii

Rajendran

et al. 2019

TH Open

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Introduction Venous thrombosis is rare in the setting of factor VIII (FVIII) deficiency. Cases of deep vein thrombosis (DVT) have been described in hemophiliacs after recent major surgery, or in association with the administration of FVIII concentrate and activated prothrombin complex concentrates, but occurrence of spontaneous DVT is even more uncommon. Aim We describe the challenging management of extensive DVT in a patient with acquired hemophilia A with concurrent hemorrhagic manifestations and review similar published cases. Methods We summarize a series of 10 cases with the following demographics: 6 males and 4 females; median age at presentation of 65 (21–80); mean inhibitor titer of 68.5 Bethesda Units (BU 1.9 to BU 350). Results Four cases were idiopathic and six had associated conditions (cancer [two cases], recent pregnancy [two cases], and recent surgery [two cases]). Three cases had an inferior vena cava filter inserted for acute lower limb DVT/pulmonary embolism. Inhibitor eradication was achieved with high-dose steroids with or without cyclophosphamide, and adjunct Rituximab administration was used in three cases. One patient received concurrent therapeutic plasma exchange (TPE). Inhibitor eradication was fastest with concurrent TPE at 6 days (range: 6–733 days). The 30-day survival was 90%. Conclusions There was adequate response of inhibitors to immunosuppression with steroids and cyclophosphamide therapy. For more refractory disease, Rituximab is emerging as a beneficial and cost-effective adjunct with better rates of complete remission, and the threshold for its use may be lowered in this complex cohort with dual competing pathologies.

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Sequential thrombosis and bleeding in a woman with a prolonged activated partial thromboplastin time

Cited by 6 publications

References 30 publications

Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report

Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report

Prolonged Activated Partial Thromboplastin Time

Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies

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