An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation

Xavier Jaïs; Stephen J Till; Thérèse Cynober; Vincent Ioos; Gilles Garcia; Gil Tchernia; Philippe Dartevelle; Gérald Simonneau; Jean Delaunay; Marc Humbert

doi:10.1081/HEM-120023377

An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation

Xavier Jaïs, Stephen J Till, Thérèse Cynober, Vincent Ioos, Gilles Garcia, Gil Tchernia, Philippe Dartevelle, Gérald Simonneau, Jean Delaunay, Marc Humbert

Research output: Contribution to journal › Article › peer-review

62 Citations (Scopus)

Abstract

Dehydrated hereditary stomatocytosis (DHS) belongs to the heterogeneous class of hemolytic anemias with leaky red cell membranes. Splenectomy is a highly deleterious treatment, because it favors, with virtually no exception, the occurrence of thromboembolic disease. We describe here the extreme case of a patient with DHS and an associated sickle cell trait. Splenectomy was carried out due to a splenic infarction that occurred during an airplane journey. About 12 years later, the patient noticed an exertional dyspnea, which gradually worsened to such a degree that she became severely incapacitated within 5 years. Eventually, the patient developed a cor pulmonale associated with chronic thromboembolic pulmonary hypertension (CTEPH) and successfully underwent a heart-lung transplant operation. This case ranks as one of the most severe examples ever recorded of the effect that splenectomy may have in DHS patients. Nonetheless, it represents the first case to receive a heart-lung transplant.

Original language	English
Pages (from-to)	139-47
Number of pages	9
Journal	Hemoglobin
Volume	27
Issue number	3
DOIs	https://doi.org/10.1081/HEM-120023377
Publication status	Published - Aug 2003

Keywords

Anemia, Hemolytic, Congenital
Pulmonary Embolism
Heart-Lung Transplantation
Splenectomy
Hypertension, Pulmonary
Humans
Adult
Treatment Outcome
Dyspnea
Anemia, Sickle Cell
Female

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1081/HEM-120023377

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title = "An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation",

abstract = "Dehydrated hereditary stomatocytosis (DHS) belongs to the heterogeneous class of hemolytic anemias with leaky red cell membranes. Splenectomy is a highly deleterious treatment, because it favors, with virtually no exception, the occurrence of thromboembolic disease. We describe here the extreme case of a patient with DHS and an associated sickle cell trait. Splenectomy was carried out due to a splenic infarction that occurred during an airplane journey. About 12 years later, the patient noticed an exertional dyspnea, which gradually worsened to such a degree that she became severely incapacitated within 5 years. Eventually, the patient developed a cor pulmonale associated with chronic thromboembolic pulmonary hypertension (CTEPH) and successfully underwent a heart-lung transplant operation. This case ranks as one of the most severe examples ever recorded of the effect that splenectomy may have in DHS patients. Nonetheless, it represents the first case to receive a heart-lung transplant.",

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author = "Xavier Ja{\"i}s and Till, {Stephen J} and Th{\'e}r{\`e}se Cynober and Vincent Ioos and Gilles Garcia and Gil Tchernia and Philippe Dartevelle and G{\'e}rald Simonneau and Jean Delaunay and Marc Humbert",

year = "2003",

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doi = "10.1081/HEM-120023377",

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T1 - An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis

T2 - gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation

AU - Jaïs, Xavier

AU - Till, Stephen J

AU - Cynober, Thérèse

AU - Ioos, Vincent

AU - Garcia, Gilles

AU - Tchernia, Gil

AU - Dartevelle, Philippe

AU - Simonneau, Gérald

AU - Delaunay, Jean

AU - Humbert, Marc

PY - 2003/8

Y1 - 2003/8

N2 - Dehydrated hereditary stomatocytosis (DHS) belongs to the heterogeneous class of hemolytic anemias with leaky red cell membranes. Splenectomy is a highly deleterious treatment, because it favors, with virtually no exception, the occurrence of thromboembolic disease. We describe here the extreme case of a patient with DHS and an associated sickle cell trait. Splenectomy was carried out due to a splenic infarction that occurred during an airplane journey. About 12 years later, the patient noticed an exertional dyspnea, which gradually worsened to such a degree that she became severely incapacitated within 5 years. Eventually, the patient developed a cor pulmonale associated with chronic thromboembolic pulmonary hypertension (CTEPH) and successfully underwent a heart-lung transplant operation. This case ranks as one of the most severe examples ever recorded of the effect that splenectomy may have in DHS patients. Nonetheless, it represents the first case to receive a heart-lung transplant.

AB - Dehydrated hereditary stomatocytosis (DHS) belongs to the heterogeneous class of hemolytic anemias with leaky red cell membranes. Splenectomy is a highly deleterious treatment, because it favors, with virtually no exception, the occurrence of thromboembolic disease. We describe here the extreme case of a patient with DHS and an associated sickle cell trait. Splenectomy was carried out due to a splenic infarction that occurred during an airplane journey. About 12 years later, the patient noticed an exertional dyspnea, which gradually worsened to such a degree that she became severely incapacitated within 5 years. Eventually, the patient developed a cor pulmonale associated with chronic thromboembolic pulmonary hypertension (CTEPH) and successfully underwent a heart-lung transplant operation. This case ranks as one of the most severe examples ever recorded of the effect that splenectomy may have in DHS patients. Nonetheless, it represents the first case to receive a heart-lung transplant.

KW - Anemia, Hemolytic, Congenital

KW - Pulmonary Embolism

KW - Heart-Lung Transplantation

KW - Splenectomy

KW - Hypertension, Pulmonary

KW - Humans

KW - Adult

KW - Treatment Outcome

KW - Dyspnea

KW - Anemia, Sickle Cell

KW - Female

U2 - 10.1081/HEM-120023377

DO - 10.1081/HEM-120023377

M3 - Article

C2 - 12908798

SN - 0363-0269

VL - 27

SP - 139

EP - 147

JO - Hemoglobin

JF - Hemoglobin

IS - 3

ER -

An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation

Abstract

Keywords

UN SDGs

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