Pulmonary hypertension in sickle cell hemoglobinopathy

A clinicopathologic study of 20 cases

Abida K. Haque, Sumita Gokhale, Bill A. Rampy, Patrick Adegboyega, Alexander Duarte, Mario J. Saldana

Research output: Contribution to journalArticle

154 Citations (Scopus)

Abstract

Pulmonary hypertension is one of the major causes of morbidity and mortality of patients with sickle cell hemoglobinopathy (SCH). Although a clinically recognized complication of sickle cell disease (SCD), there are few published pathologic studies of pulmonary findings in these patients. The aim of this study was to define the pulmonary pathologic changes and to investigate correlation between the pathologic changes, the antemortem diagnosis of pulmonary hypertension, and the severity of SCH. Cases of SCH were identified from the autopsy database using Snomed codes. Clinical and echocardiograph data were collected for correlation with the pathologic data. A total of 20 adult patients (12 males and 8 females) were identified. Hemoglobin electrophoresis results were available for 16 patients, with hemoglobin S fraction percentages ranging from 23% to 97.8%. Eleven patients had SCD, 5 patients had sickle cell trait (SCT), and the remaining 4 patients without hemoglobin electrophoresis were included in the SCT group. The mean age of the SCT group was higher than that of the SCD group (P = 0.03). Histologically, all 20 patients demonstrated changes in pulmonary vasculature considered diagnostic of pulmonary hypertension grade I to grade IV, associated with plexiform lesions in 60% of patients. Medial hypertrophy and intimal hyperplasia/fibrosis, considered potentially reversible lesions, were seen in all patients. A weak association was fotmd between SCD and plexiform lesions. Fibroelastic degeneration of small arteries, arterioles, and venules was identified in almost all (95%) cases. Clinically, tricuspid regurgitation was detected by echocardiogram in 10 of 20 (50%) patients; 6 of these 10 had significant regurgitation to allow estimation of systolic pressure. Sudden death occurred in 8 patients, with males having a significantly higher incidence. Cardiomegaly was present in 95% of patients, however, autosplenectomy and hepatic cirrhosis/hemochromatosis were observed almost exclusively in patients with SCD. Cirrhosis was found to have a strong positive association with SCD. This study demonstrates pulmonary hypertensive changes in all 20 autopsied patients who had SCH but died from various causes. We conclude that a high prevalence of pulmonary hypertension is associated with SCH with consequent high mortality. Therefore, patients with SCH would benefit from a regular periodic assessment for pulmonary hypertension regardless of age, sex, and severity of hemoglobinopathy.

Original languageEnglish (US)
Pages (from-to)1037-1043
Number of pages7
JournalHuman Pathology
Volume33
Issue number10
DOIs
StatePublished - Oct 1 2002

Fingerprint

Hemoglobinopathies
Pulmonary Hypertension
Sickle Cell Anemia
Sickle Cell Trait
Lung
Electrophoresis
Hemoglobins
Fibrosis
Tunica Intima
Sickle Hemoglobin
Tricuspid Valve Insufficiency
Hemochromatosis
Venules
Mortality
Cardiomegaly
Arterioles
Sudden Death

Keywords

  • Lung pathology
  • Pulmonary hypertension
  • Sickle cell hemoglobinopathy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Pulmonary hypertension in sickle cell hemoglobinopathy : A clinicopathologic study of 20 cases. / Haque, Abida K.; Gokhale, Sumita; Rampy, Bill A.; Adegboyega, Patrick; Duarte, Alexander; Saldana, Mario J.

In: Human Pathology, Vol. 33, No. 10, 01.10.2002, p. 1037-1043.

Research output: Contribution to journalArticle

Haque, AK, Gokhale, S, Rampy, BA, Adegboyega, P, Duarte, A & Saldana, MJ 2002, 'Pulmonary hypertension in sickle cell hemoglobinopathy: A clinicopathologic study of 20 cases', Human Pathology, vol. 33, no. 10, pp. 1037-1043. https://doi.org/10.1053/hupa.2002.128059
Haque, Abida K. ; Gokhale, Sumita ; Rampy, Bill A. ; Adegboyega, Patrick ; Duarte, Alexander ; Saldana, Mario J. / Pulmonary hypertension in sickle cell hemoglobinopathy : A clinicopathologic study of 20 cases. In: Human Pathology. 2002 ; Vol. 33, No. 10. pp. 1037-1043.
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abstract = "Pulmonary hypertension is one of the major causes of morbidity and mortality of patients with sickle cell hemoglobinopathy (SCH). Although a clinically recognized complication of sickle cell disease (SCD), there are few published pathologic studies of pulmonary findings in these patients. The aim of this study was to define the pulmonary pathologic changes and to investigate correlation between the pathologic changes, the antemortem diagnosis of pulmonary hypertension, and the severity of SCH. Cases of SCH were identified from the autopsy database using Snomed codes. Clinical and echocardiograph data were collected for correlation with the pathologic data. A total of 20 adult patients (12 males and 8 females) were identified. Hemoglobin electrophoresis results were available for 16 patients, with hemoglobin S fraction percentages ranging from 23{\%} to 97.8{\%}. Eleven patients had SCD, 5 patients had sickle cell trait (SCT), and the remaining 4 patients without hemoglobin electrophoresis were included in the SCT group. The mean age of the SCT group was higher than that of the SCD group (P = 0.03). Histologically, all 20 patients demonstrated changes in pulmonary vasculature considered diagnostic of pulmonary hypertension grade I to grade IV, associated with plexiform lesions in 60{\%} of patients. Medial hypertrophy and intimal hyperplasia/fibrosis, considered potentially reversible lesions, were seen in all patients. A weak association was fotmd between SCD and plexiform lesions. Fibroelastic degeneration of small arteries, arterioles, and venules was identified in almost all (95{\%}) cases. Clinically, tricuspid regurgitation was detected by echocardiogram in 10 of 20 (50{\%}) patients; 6 of these 10 had significant regurgitation to allow estimation of systolic pressure. Sudden death occurred in 8 patients, with males having a significantly higher incidence. Cardiomegaly was present in 95{\%} of patients, however, autosplenectomy and hepatic cirrhosis/hemochromatosis were observed almost exclusively in patients with SCD. Cirrhosis was found to have a strong positive association with SCD. This study demonstrates pulmonary hypertensive changes in all 20 autopsied patients who had SCH but died from various causes. We conclude that a high prevalence of pulmonary hypertension is associated with SCH with consequent high mortality. Therefore, patients with SCH would benefit from a regular periodic assessment for pulmonary hypertension regardless of age, sex, and severity of hemoglobinopathy.",
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