Paraneoplastic acquired haemophilia A in extensive-stage small cell lung cancer (ES-SCLC) in the era of immunotherapy

BMJ Case Rep. 2021 Jan 28;14(1):e236973. doi: 10.1136/bcr-2020-236973.

Abstract

Small cell lung cancer (SCLC) is a deadly and rapidly progressive disease that can present with various paraneoplastic syndromes on initial workup. Acquired factor VIII (FVIII) deficiency, also known as acquired haemophilia A (AHA), has been identified as a rare paraneoplastic syndrome in SCLC. Here, we present a 61-year-old woman with a massive gastrointestinal bleed and prolonged activated partial thromboplastin time (PTT) in the emergency department. She was diagnosed with rare paraneoplastic AHA secondary to extensive-stage SCLC (ES-SCLC). She was treated with high-dose steroids and factor bypassing agents, which led to the resolution of bleeding and undetectable FVIII inhibitor levels. She was subsequently treated for ES-SCLC with carboplatin, etoposide and atezolizumab. This case report highlights a rare clinical presentation of paraneoplastic AHA that necessitates prompt recognition in patients with SCLC with ongoing bleeding and elevated PTT.

Keywords: GI bleeding; haematology (incl blood transfusion); lung cancer (oncology).

Publication types

  • Case Reports

MeSH terms

  • Abdomen
  • Adrenal Gland Neoplasms / complications
  • Adrenal Gland Neoplasms / diagnosis*
  • Adrenal Gland Neoplasms / drug therapy
  • Adrenal Gland Neoplasms / secondary
  • Anemia / diagnosis
  • Anemia / etiology
  • Antineoplastic Agents / therapeutic use
  • Autoantibodies / immunology
  • Coagulants / therapeutic use
  • Erythrocyte Transfusion
  • Factor VIII / immunology
  • Factor VIII / therapeutic use
  • Factor VIIa / therapeutic use
  • Female
  • Gastrointestinal Hemorrhage / diagnosis*
  • Gastrointestinal Hemorrhage / etiology
  • Hemophilia A / diagnosis*
  • Hemophilia A / etiology
  • Hemophilia A / immunology
  • Hemophilia A / therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung Neoplasms / complications
  • Lung Neoplasms / diagnosis*
  • Lung Neoplasms / pathology
  • Lymphadenopathy
  • Mediastinum
  • Middle Aged
  • Paraneoplastic Syndromes / diagnosis*
  • Paraneoplastic Syndromes / etiology
  • Paraneoplastic Syndromes / immunology
  • Paraneoplastic Syndromes / therapy
  • Small Cell Lung Carcinoma / complications
  • Small Cell Lung Carcinoma / diagnosis*
  • Small Cell Lung Carcinoma / drug therapy
  • Small Cell Lung Carcinoma / secondary

Substances

  • Antineoplastic Agents
  • Autoantibodies
  • Coagulants
  • Immunosuppressive Agents
  • Factor VIII
  • Factor VIIa

Supplementary concepts

  • Factor 8 deficiency, acquired