Sickle cell anemia with systemic lupus erythematosus

Response to hydroxyurea therapy

Avinash K. Shetty, S. Rajeev Kumar, Abraham Gedalia, Raj P. Warrier

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Purpose: To report the efficacy of hydroxyurea (HU) in a patient with sickle cell anemia (SCA) associated with systemic lupus erythematosus (SLE). Patients and Methods: An unusual association of SCA with SLE occurred in a 14-year-old black girl. Her medical history was significant for repeated vasoocclusive crises requiring repeated hospitalizations and transfusions, acute chest syndrome, hyperhemolytic crises, multiple transfusions resulting in iron overload, gall stones, avascular necrosis, and significant psychosocial problems. Her lupus activity was controlled with oral prednisone and hydroxychloroquine. Treatment with oral HU was instituted at an initial dose of 20 mg/kg with gradual increment to a maximum of 25 mg/kg daily. Results: A dramatic clinical improvement was noted with HU therapy with no episode of pain crisis or hospitalization for 5 months. There was an increase in fetal hemoglobin (HbF) to 20.9% and mean corpuscular volume to 114.5 fl, but her Hb level remained steady at 7.5 g/dl. No toxicity was noted with HU therapy. In addition, a significant change was also observed in her school performance, social activities, and general quality of life. Conclusions: HU therapy may be beneficial and safe and should be considered for other patients who have SCA with SLE.

Original languageEnglish
Pages (from-to)335-337
Number of pages3
JournalJournal of Pediatric Hematology/Oncology
Volume20
Issue number4
DOIs
Publication statusPublished - 01-07-1998
Externally publishedYes

Fingerprint

Hydroxyurea
Sickle Cell Anemia
Systemic Lupus Erythematosus
Hospitalization
Acute Chest Syndrome
Hydroxychloroquine
Therapeutics
Fetal Hemoglobin
Iron Overload
Erythrocyte Indices
Gallstones
Prednisone
Necrosis
Quality of Life
Pain

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Shetty, Avinash K. ; Kumar, S. Rajeev ; Gedalia, Abraham ; Warrier, Raj P. / Sickle cell anemia with systemic lupus erythematosus : Response to hydroxyurea therapy. In: Journal of Pediatric Hematology/Oncology. 1998 ; Vol. 20, No. 4. pp. 335-337.
@article{865c5054a693431e89d0348714e643dc,
title = "Sickle cell anemia with systemic lupus erythematosus: Response to hydroxyurea therapy",
abstract = "Purpose: To report the efficacy of hydroxyurea (HU) in a patient with sickle cell anemia (SCA) associated with systemic lupus erythematosus (SLE). Patients and Methods: An unusual association of SCA with SLE occurred in a 14-year-old black girl. Her medical history was significant for repeated vasoocclusive crises requiring repeated hospitalizations and transfusions, acute chest syndrome, hyperhemolytic crises, multiple transfusions resulting in iron overload, gall stones, avascular necrosis, and significant psychosocial problems. Her lupus activity was controlled with oral prednisone and hydroxychloroquine. Treatment with oral HU was instituted at an initial dose of 20 mg/kg with gradual increment to a maximum of 25 mg/kg daily. Results: A dramatic clinical improvement was noted with HU therapy with no episode of pain crisis or hospitalization for 5 months. There was an increase in fetal hemoglobin (HbF) to 20.9{\%} and mean corpuscular volume to 114.5 fl, but her Hb level remained steady at 7.5 g/dl. No toxicity was noted with HU therapy. In addition, a significant change was also observed in her school performance, social activities, and general quality of life. Conclusions: HU therapy may be beneficial and safe and should be considered for other patients who have SCA with SLE.",
author = "Shetty, {Avinash K.} and Kumar, {S. Rajeev} and Abraham Gedalia and Warrier, {Raj P.}",
year = "1998",
month = "7",
day = "1",
doi = "10.1097/00043426-199807000-00011",
language = "English",
volume = "20",
pages = "335--337",
journal = "Journal of Pediatric Hematology/Oncology",
issn = "1077-4114",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

Sickle cell anemia with systemic lupus erythematosus : Response to hydroxyurea therapy. / Shetty, Avinash K.; Kumar, S. Rajeev; Gedalia, Abraham; Warrier, Raj P.

In: Journal of Pediatric Hematology/Oncology, Vol. 20, No. 4, 01.07.1998, p. 335-337.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Sickle cell anemia with systemic lupus erythematosus

T2 - Response to hydroxyurea therapy

AU - Shetty, Avinash K.

AU - Kumar, S. Rajeev

AU - Gedalia, Abraham

AU - Warrier, Raj P.

PY - 1998/7/1

Y1 - 1998/7/1

N2 - Purpose: To report the efficacy of hydroxyurea (HU) in a patient with sickle cell anemia (SCA) associated with systemic lupus erythematosus (SLE). Patients and Methods: An unusual association of SCA with SLE occurred in a 14-year-old black girl. Her medical history was significant for repeated vasoocclusive crises requiring repeated hospitalizations and transfusions, acute chest syndrome, hyperhemolytic crises, multiple transfusions resulting in iron overload, gall stones, avascular necrosis, and significant psychosocial problems. Her lupus activity was controlled with oral prednisone and hydroxychloroquine. Treatment with oral HU was instituted at an initial dose of 20 mg/kg with gradual increment to a maximum of 25 mg/kg daily. Results: A dramatic clinical improvement was noted with HU therapy with no episode of pain crisis or hospitalization for 5 months. There was an increase in fetal hemoglobin (HbF) to 20.9% and mean corpuscular volume to 114.5 fl, but her Hb level remained steady at 7.5 g/dl. No toxicity was noted with HU therapy. In addition, a significant change was also observed in her school performance, social activities, and general quality of life. Conclusions: HU therapy may be beneficial and safe and should be considered for other patients who have SCA with SLE.

AB - Purpose: To report the efficacy of hydroxyurea (HU) in a patient with sickle cell anemia (SCA) associated with systemic lupus erythematosus (SLE). Patients and Methods: An unusual association of SCA with SLE occurred in a 14-year-old black girl. Her medical history was significant for repeated vasoocclusive crises requiring repeated hospitalizations and transfusions, acute chest syndrome, hyperhemolytic crises, multiple transfusions resulting in iron overload, gall stones, avascular necrosis, and significant psychosocial problems. Her lupus activity was controlled with oral prednisone and hydroxychloroquine. Treatment with oral HU was instituted at an initial dose of 20 mg/kg with gradual increment to a maximum of 25 mg/kg daily. Results: A dramatic clinical improvement was noted with HU therapy with no episode of pain crisis or hospitalization for 5 months. There was an increase in fetal hemoglobin (HbF) to 20.9% and mean corpuscular volume to 114.5 fl, but her Hb level remained steady at 7.5 g/dl. No toxicity was noted with HU therapy. In addition, a significant change was also observed in her school performance, social activities, and general quality of life. Conclusions: HU therapy may be beneficial and safe and should be considered for other patients who have SCA with SLE.

UR - http://www.scopus.com/inward/record.url?scp=0031729245&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0031729245&partnerID=8YFLogxK

U2 - 10.1097/00043426-199807000-00011

DO - 10.1097/00043426-199807000-00011

M3 - Article

VL - 20

SP - 335

EP - 337

JO - Journal of Pediatric Hematology/Oncology

JF - Journal of Pediatric Hematology/Oncology

SN - 1077-4114

IS - 4

ER -