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Fragmentation hemolysis in a patient with hypertrophic obstructive cardiomyopathy and mitral valve prolapse
1Department of Internal Medicine, Nippon Steel Corporation Muroran Works Hospital, Muroran, Japan.
Insights
A patient with hypertrophic obstructive cardiomyopathy experienced hemolytic anemia due to group A streptococci endocarditis. Red blood cell fragmentation, likely from shear stress, improved with propranolol treatment.
Area of Science:
- Cardiology
- Hematology
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) is a significant cardiac condition.
- Infective endocarditis can lead to various systemic complications.
- Hemolytic anemia presents a diagnostic challenge, especially when co-occurring with cardiac conditions.
Observation:
- A 65-year-old female with HOCM and mitral valve prolapse developed infective endocarditis caused by group A streptococci.
- The patient presented with hemolytic anemia, characterized by fragmented red blood cells, decreased haptoglobin, and elevated lactic dehydrogenase.
- Cardiac investigations revealed significant left ventricular pressure gradients and mitral regurgitation.
Findings:
- Fragmentative hemolysis was identified as the cause of anemia, with negative Coombs' test, Ham's test, and normal hemoglobin electrophoresis.
- Shear stress within the left ventricle was implicated as the mechanism for red blood cell fragmentation.
- Hemolysis showed slight improvement following propranolol therapy.
Implications:
- This case highlights the complex interplay between hypertrophic obstructive cardiomyopathy, infective endocarditis, and secondary hemolytic anemia.
- Shear stress induced by pressure gradients in HOCM can cause fragmentation hemolysis.
- Management of the underlying cardiac condition and associated factors is crucial for improving hematological parameters.
Abstract:
We encountered a 65-year-old female with hypertrophic obstructive cardiomyopathy and mitral valve prolapse who had infective endocarditis and hemolytic anemia. The infecting organism of endocarditis was group A streptococci. With regard to the etiology of the hemolytic anemia, fragmentation hemolysis was considered because fragmented red cells and elevated lactic dehydrogenase were observed. Haptoglobin was markedly decreased. Coombs' test, Ham's test and abnormal hemoglobin were negative. She had not had a hemolytic attack in the past. Ultrasonic cardiography showed asymmetrical septal hypertrophy, mitral valve prolapse and 285 mmHg of calculated pressure gradient in the left ventricle. Cardiac catheterization showed 115 mmHg of left intraventricular pressure gradient and mitral regurgitation (grade 2). Hemolysis was slightly improved after treatment with propranolol. Thus, fragmentation of the normal red cells seemed to be due to shear stress.