Acute splenic sequestration crisis in adult sickle cell disease: A report of 16 cases

Leonard Naymagon, Gopichand Pendurti, Henny H. Billett

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Acute splenic sequestration crisis (ASSC), characterized by rapidly progressive anemia and circulatory compromise in the setting of sudden splenic enlargement, is an uncommon entity among adult sickle cell patients. We reviewed cases of adult ASSC encountered at our institution to generate insight into the recognition, diagnosis, and treatment of the condition. Cases of adult ASSC during a 10-year period were identified retrospectively. Patient charts were reviewed for laboratory and imaging results; demographic data and clinical course were collected and reviewed. Sixteen cases of adult ASSC were identified. Most patients presented with pain crisis; only four of 16 patients presented with abdominal pain. The maximum decreases in hemoglobin (Hb) (42.0%) and platelets (62.1%) occurred at day 2.9, delaying identification and treatment. Hemodynamic instability played a large role in dictating risk stratification. Therapy consisted of transfusion (14/16) and splenectomy (5/16). No recurrences were noted in a mean follow-up time of 5.3 years but review of patients charts demonstrated that at least one of the patients had two prior episodes. Adult ASSC may present with non specific findings and patients may not deteriorate until several days into a previously uneventful hospital course. Changes in platelet counts may be more reliable markers than changes in Hb level since red cell transfusions may interfere with assessments of the sequestration process. This case series of adult ASSC, the largest reported in the literature to date, highlights common clinical, laboratory, radiological, and pathological features of this uncommon entity and helps to guide recognition, diagnosis, and treatment.

Original languageEnglish (US)
Pages (from-to)375-379
Number of pages5
JournalHemoglobin
Volume39
Issue number6
DOIs
StatePublished - Nov 2 2015

Fingerprint

Sickle Cell Anemia
Platelets
Hemoglobins
Clinical laboratories
Hemodynamics
Cells
Imaging techniques
Process Assessment (Health Care)
Splenectomy
Therapeutics
Platelet Count
Abdominal Pain
Anemia
Blood Platelets
Demography
Recurrence
Pain

Keywords

  • Acute splenic sequestration crisis (ASSC)
  • sickle cell disease
  • splenomegaly

ASJC Scopus subject areas

  • Hematology
  • Genetics(clinical)
  • Clinical Biochemistry
  • Biochemistry, medical

Cite this

Acute splenic sequestration crisis in adult sickle cell disease : A report of 16 cases. / Naymagon, Leonard; Pendurti, Gopichand; Billett, Henny H.

In: Hemoglobin, Vol. 39, No. 6, 02.11.2015, p. 375-379.

Research output: Contribution to journalArticle

Naymagon, Leonard ; Pendurti, Gopichand ; Billett, Henny H. / Acute splenic sequestration crisis in adult sickle cell disease : A report of 16 cases. In: Hemoglobin. 2015 ; Vol. 39, No. 6. pp. 375-379.
@article{2cf6cef396cc4bb39f98d2f0c2c39166,
title = "Acute splenic sequestration crisis in adult sickle cell disease: A report of 16 cases",
abstract = "Acute splenic sequestration crisis (ASSC), characterized by rapidly progressive anemia and circulatory compromise in the setting of sudden splenic enlargement, is an uncommon entity among adult sickle cell patients. We reviewed cases of adult ASSC encountered at our institution to generate insight into the recognition, diagnosis, and treatment of the condition. Cases of adult ASSC during a 10-year period were identified retrospectively. Patient charts were reviewed for laboratory and imaging results; demographic data and clinical course were collected and reviewed. Sixteen cases of adult ASSC were identified. Most patients presented with pain crisis; only four of 16 patients presented with abdominal pain. The maximum decreases in hemoglobin (Hb) (42.0{\%}) and platelets (62.1{\%}) occurred at day 2.9, delaying identification and treatment. Hemodynamic instability played a large role in dictating risk stratification. Therapy consisted of transfusion (14/16) and splenectomy (5/16). No recurrences were noted in a mean follow-up time of 5.3 years but review of patients charts demonstrated that at least one of the patients had two prior episodes. Adult ASSC may present with non specific findings and patients may not deteriorate until several days into a previously uneventful hospital course. Changes in platelet counts may be more reliable markers than changes in Hb level since red cell transfusions may interfere with assessments of the sequestration process. This case series of adult ASSC, the largest reported in the literature to date, highlights common clinical, laboratory, radiological, and pathological features of this uncommon entity and helps to guide recognition, diagnosis, and treatment.",
keywords = "Acute splenic sequestration crisis (ASSC), sickle cell disease, splenomegaly",
author = "Leonard Naymagon and Gopichand Pendurti and Billett, {Henny H.}",
year = "2015",
month = "11",
day = "2",
doi = "10.3109/03630269.2015.1072550",
language = "English (US)",
volume = "39",
pages = "375--379",
journal = "Hemoglobin",
issn = "0363-0269",
publisher = "Informa Healthcare",
number = "6",

}

TY - JOUR

T1 - Acute splenic sequestration crisis in adult sickle cell disease

T2 - A report of 16 cases

AU - Naymagon, Leonard

AU - Pendurti, Gopichand

AU - Billett, Henny H.

PY - 2015/11/2

Y1 - 2015/11/2

N2 - Acute splenic sequestration crisis (ASSC), characterized by rapidly progressive anemia and circulatory compromise in the setting of sudden splenic enlargement, is an uncommon entity among adult sickle cell patients. We reviewed cases of adult ASSC encountered at our institution to generate insight into the recognition, diagnosis, and treatment of the condition. Cases of adult ASSC during a 10-year period were identified retrospectively. Patient charts were reviewed for laboratory and imaging results; demographic data and clinical course were collected and reviewed. Sixteen cases of adult ASSC were identified. Most patients presented with pain crisis; only four of 16 patients presented with abdominal pain. The maximum decreases in hemoglobin (Hb) (42.0%) and platelets (62.1%) occurred at day 2.9, delaying identification and treatment. Hemodynamic instability played a large role in dictating risk stratification. Therapy consisted of transfusion (14/16) and splenectomy (5/16). No recurrences were noted in a mean follow-up time of 5.3 years but review of patients charts demonstrated that at least one of the patients had two prior episodes. Adult ASSC may present with non specific findings and patients may not deteriorate until several days into a previously uneventful hospital course. Changes in platelet counts may be more reliable markers than changes in Hb level since red cell transfusions may interfere with assessments of the sequestration process. This case series of adult ASSC, the largest reported in the literature to date, highlights common clinical, laboratory, radiological, and pathological features of this uncommon entity and helps to guide recognition, diagnosis, and treatment.

AB - Acute splenic sequestration crisis (ASSC), characterized by rapidly progressive anemia and circulatory compromise in the setting of sudden splenic enlargement, is an uncommon entity among adult sickle cell patients. We reviewed cases of adult ASSC encountered at our institution to generate insight into the recognition, diagnosis, and treatment of the condition. Cases of adult ASSC during a 10-year period were identified retrospectively. Patient charts were reviewed for laboratory and imaging results; demographic data and clinical course were collected and reviewed. Sixteen cases of adult ASSC were identified. Most patients presented with pain crisis; only four of 16 patients presented with abdominal pain. The maximum decreases in hemoglobin (Hb) (42.0%) and platelets (62.1%) occurred at day 2.9, delaying identification and treatment. Hemodynamic instability played a large role in dictating risk stratification. Therapy consisted of transfusion (14/16) and splenectomy (5/16). No recurrences were noted in a mean follow-up time of 5.3 years but review of patients charts demonstrated that at least one of the patients had two prior episodes. Adult ASSC may present with non specific findings and patients may not deteriorate until several days into a previously uneventful hospital course. Changes in platelet counts may be more reliable markers than changes in Hb level since red cell transfusions may interfere with assessments of the sequestration process. This case series of adult ASSC, the largest reported in the literature to date, highlights common clinical, laboratory, radiological, and pathological features of this uncommon entity and helps to guide recognition, diagnosis, and treatment.

KW - Acute splenic sequestration crisis (ASSC)

KW - sickle cell disease

KW - splenomegaly

UR - http://www.scopus.com/inward/record.url?scp=84945469728&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84945469728&partnerID=8YFLogxK

U2 - 10.3109/03630269.2015.1072550

DO - 10.3109/03630269.2015.1072550

M3 - Article

C2 - 26287797

AN - SCOPUS:84945469728

VL - 39

SP - 375

EP - 379

JO - Hemoglobin

JF - Hemoglobin

SN - 0363-0269

IS - 6

ER -