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Pulmonary Tumor Thrombotic Microangiopathy as a Cause of Pulmonary Hypertension.

ABSTRACT: Pulmonary tumor thrombotic microangiopathy is a rare entity, often diagnosed postmortem. We describe a patient with signs and symptoms of pulmonary hypertension secondary to metastatic cholangiocarcinoma with invasion of the myocardium and pulmonary vasculature, and highlight the diagnostic challenges and therapeutic limitations of this disease. (Level of Difficulty: Intermediate.).

SUBMITTER: Schwartz BP

PROVIDER: S-EPMC8311364 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature

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Pulmonary Tumor Thrombotic Microangiopathy as a Cause of Pulmonary Hypertension.

Schwartz Benjamin P BP Tracy Philip P Hon Stephanie S Farber Harrison W HW Udelson James E JE

JACC. Case reports 20210707 7

Pulmonary tumor thrombotic microangiopathy is a rare entity, often diagnosed postmortem. We describe a patient with signs and symptoms of pulmonary hypertension secondary to metastatic cholangiocarcinoma with invasion of the myocardium and pulmonary vasculature, and highlight the diagnostic challenges and therapeutic limitations of this disease. (<b>Level of Difficulty: Intermediate.</b>). ...[more]

PMID: 34317678

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Project description:BackgroundLipoprotein glomerulopathy (LPG) is a rare inherited renal disease characterized by intraglomerular lipoprotein within the lumina of severely dilated glomerular capillaries. The common clinical presentation of LPG includes proteinuria or nephrotic syndrome. Hypertension and anemia were thought to be mild in LPG. Thrombotic microangiopathy (TMA) in LPG has not been previously reported. In this report, we present a patient with LPG that developed TMA. To the best of our knowledge, this is the first report of TMA in LPG.Case presentationFour years ago (2005), a 19-year-old Chinese woman was diagnosed with nephrotic syndrome and provided prednisone treatment. A combination of prednisone and cyclophosphamide did not have any effect and was discontinued after six months. Although she was steroid-resistant, over the next subsequent three years, she maintained normal renal function without anemia and thrombocytopenia. In February 2009, she had a severe headache and blurry vision and presented at a local hospital with severe hypertension. Blood pressure was 220/160 mmHg. Laboratory data showed hemoglobin 3.8 g/dL; platelet counts 29 × 10(9)/L; urinary protein 7.90 g/d; total bilirubin 29.9 umol/L; indirect bilirubin 28.2 umol/L; LDH 1172 U/L; ALB 2.66 g/dL; urea nitrogen 52 mg/dL; serum creatinine 3.2 mg/dL; triglyceride 25 mg/dL; total cholesterol 273 mg/dL. ANA, ds-DNA, ANCA, anti-GBM antibody and anticardiolipin were all negative. A renal biopsy revealed LPG with TMA. Genetic evaluation showed the patient carried the APOE Kyoto mutation. Adequate control of blood pressure improved microangiopathic anemia and thrombocytopenia, however, renal function did not improve and she eventually developed uremia and became hemodialysis dependent.ConclusionWe report on a rare case of TMA probably due to malignant hypertension in LPG. Early lipid-lowering and antihypertensive treatment may improve outcome. The pathophysiologic relationship between LPG and TMA should be investigated further.

Dataset Information

Pulmonary Tumor Thrombotic Microangiopathy as a Cause of Pulmonary Hypertension.

Publications

Pulmonary Tumor Thrombotic Microangiopathy as a Cause of Pulmonary Hypertension.

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