scholarly journals Acute Splenic Sequestration Crisis in Hemoglobin SC Disease: Efficiency of Red Cell Exchange

Cureus ◽  
2020 ◽  
Author(s):  
Anjanaa Vijayanarayanan ◽  
Ayodeji J Omosule ◽  
Hannan Saad ◽  
Vrushali Dabak ◽  
Zaher K Otrock
Keyword(s):  
Red Cell ◽  
Splenic Sequestration ◽  
Hemoglobin Sc Disease

Case Report: Splenic Infarction and Acute Splenic Sequestration in Adults with Hemoglobin SC Disease

1991 ◽  
Vol 302 (6) ◽  
pp. 374-379 ◽  
Author(s):  
Eugene P. Orringer ◽  
Vance G. Fowler ◽  
Catrell M. Owens ◽  
Adrena E. Johnson ◽  
Matthew A. Mauro ◽  
...  
Keyword(s):  
Case Report ◽  
Splenic Infarction ◽  
Splenic Sequestration ◽  
Hemoglobin Sc Disease

scholarly journals Recurrent acute splenic sequestration crisis due to interacting genetic defects: hemoglobin SC disease and hereditary spherocytosis

Blood ◽  
1990 ◽  
Vol 75 (1) ◽  
pp. 266-270 ◽  
Author(s):  
TE Warkentin ◽  
RD Barr ◽  
MA Ali ◽  
N Mohandas
Keyword(s):  
Hereditary Spherocytosis ◽  
Genetic Defects ◽  
Mechanical Instability ◽  
Splenic Sequestration ◽  
Alpha Thalassemia ◽  
Unusual Occurrence ◽  
Distinguishing Features ◽  
Hemoglobin Sc Disease ◽  
Reduced Surface ◽  
In Cells

Abstract A 14-year-old boy with hemoglobin SC disease and alpha-thalassemia-2 experienced five episodes of acute splenic sequestration crisis (ASSC), while two of his siblings with identical globin genotypes (SC and - alpha/alpha alpha) had no such experience. To determine if an additional red blood cell (RBC) defect was responsible for the unusual occurrence of frequent ASSCs, we performed detailed rheologic characterization and membrane protein analysis on RBCs from the proband and other members of his family. Reduced surface area, increased mechanical instability, and decreased spectrin content of the membrane, distinguishing features of RBCs in hereditary spherocytosis, were observed in cells from the proband and his mother, but not in cells from other family members. These findings are consistent with the dominant inheritance of spherocytosis by the proband. We suggest that the combined effects of SC disease and spherocytosis in the proband resulted in decreased RBC deformability and led to increased splenic trapping, intrasplenic sickling, and consequently, recurrent sequestration crisis. Marked clinical and hematologic improvement occurred from splenectomy. Thus, inheritance of interacting genetic defects, sickling hemoglobinopathy, and hereditary spherocytosis appear to be responsible for the unusual clinical manifestation of recurrent ASSC in this patient.

Acute Splenic Sequestration Crisis Resembling Sepsis in an Adult with Hemoglobin SC Disease

2004 ◽  
Vol 97 (4) ◽  
pp. 413-415 ◽  
Author(s):  
Andrea Wang-Gillam ◽  
Rita Shi-Ming Lee ◽  
Eric D. Hsi ◽  
Daniel J. Brotman
Keyword(s):  
Splenic Sequestration ◽  
Hemoglobin Sc Disease

scholarly journals Studies of the Production and Life Span of Erythrocytes in Myeloid Metaplasia

Blood ◽  
1959 ◽  
Vol 14 (6) ◽  
pp. 668-682 ◽  
Author(s):  
DAVID G. NATHAN ◽  
NATHANIEL I. BERLIN
Keyword(s):  
Life Span ◽  
Polycythemia Vera ◽  
Red Cell ◽  
Myeloid Metaplasia ◽  
Splenic Sequestration ◽  
Cell Life ◽  
Agnogenic Myeloid Metaplasia

Abstract 1. Four patients with agnogenic myeloid metaplasia and one patient with polycythemia vera and myeloid metaplasia were studied with Fe59, Cr51 and glycine-2-C14. 2. Three of the patients with agnogenic myeloid metaplasia had active splenic, hepatic or renal erythropoiesis. One had deficient erythropoiesis. 3. The red cell life span was short in all of the patients with agnogenic myeloid metaplasia, definite splenic sequestration occurring in two patients. The red cell life span was normal in the patient with polycythemia vera and myeloid metaplasia. 4. The possible indications for splenectomy were discussed.

Children with Sickle Cell Disease on Chronic Red Cell Transfusion Experience Fewer Hospitalizations for Acute Vaso-Occlusive Episodes Irrespective of the Indication for Transfusion

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4282-4282
Author(s):  
Carmen C Wallace ◽  
Hilda Mata ◽  
Nancy J Wandersee ◽  
J. Paul Scott ◽  
Amanda M Brandow ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Admission Rate ◽  
T Test ◽  
Acute Chest Syndrome ◽  
Red Cell ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Red Cell Transfusion ◽  
The Impact

Abstract While there is strong evidence that chronic red cell transfusion is effective in preventing primary stroke and reducing the risk of recurrent stroke in sickle cell disease (SCD), it is less clear whether chronic transfusions will prevent admissions for other acute vaso-occlusive complications, including pain, priapism and/or acute chest syndrome. To our knowledge, no study to date has investigated the effect of chronic transfusion on the frequency of admissions for acute vaso-occlusive complications in children with all diagnoses of SCD and treated with chronic transfusion for a variety of indications. In addition, this study included a special focus on the effect of chronic transfusion on children who were transfused specifically for recurrent vaso-occlusive episodes. We performed a single-site retrospective chart review. We selected subjects from all children aged 0 to 19 years who were treated (lived in the Milwaukee area) and followed by the Wisconsin Sickle Cell Center at Children’s Hospital of Wisconsin from 1984 to May 2014 (n=695 subjects). Data was extracted from any individual who was enrolled in a chronic transfusion program for a minimum of six months. Data on admissions for painful vaso-occlusive crises, acute chest syndrome (ACS), other SCD complications as well as sickle diagnosis, age at time of transfusion, CBC, reticulocyte count, and percent sickle hemoglobin (HbS%) were collected for 24 months prior to onset of transfusion and for all months during transfusion until the age of 19 yrs. Unless otherwise indicated, all statistical analyses on extracted data were done by paired Student’s t-Test. We extracted data from 103 unique subjects for 108 chronic transfusion programs (as defined above); 5 subjects were chronically transfused twice, separated by at least 4 years without chronic transfusion. 55% were female; average age was 8.6 ± 5.6 (mean ± SD) years and the sickle diagnosis included 94% SS, 3% SC, 2% Sβ°-Thalassemia and 1% SD. The indication for transfusion included pain (n=31), priapism (n=6), ACS (n=5), central nervous system complications (n=37, including stroke, TIA, and abnormal TCD), splenic sequestration (n=25), pulmonary hypertension (n=2), retinopathy (n=1) and osteomyelitis (n=1). The hemoglobin level increased from a baseline of 7.6 ± 2.2 gm/dL to 9.6 ± 0.8 gm/dL during transfusion (p<0.0001, paired t-Test). HbS% was also reduced from a baseline of 84.2 ± 10.8% to 35.8 ± 0.3% during transfusion (p<0.0001). We found that rate of admissions for acute painful episodes, including priapism, dropped from 2.2 ± 2.9 admits/yr during the 24 months pre-transfusion to 1.0 ± 1.9 admits/yr during transfusion (p<0.0001). Similarly, the rate of admission for ACS decreased from 0.3 ± 0.5 admits/yr for 24 months pre-transfusion to 0.1 ± 0.3 admits/yr during transfusion (p=0.0001). Subanalyses were performed on specific indications for transfusion. For children transfused due to frequent acute vaso-occlusive complications (pain, priapism and ACS were arbitrarily included in this group), the average age at initiation of transfusion was 11.9 ± 4.4 yr, and admissions for acute painful episodes dropped from 4.0 ± 3.2 admits/yr during the 24 months pre-transfusion to 2.1 ± 2.6 admits/yr during transfusion (p=0.003). When the indication for transfusion was splenic sequestration (age 2.3±2.7 yr), the admission rate for acute painful episodes did not change (0.8±1.7 vs 0.3±0.5 admits/yr, p= 0.14). For children transfused for CNS complications (age 8.5±4.6 yr), the admission rate for pain improved from 0.9±1.3 to 0.2±0.5 admissions/yr (p=0.007). In agreement with previous studies, our data also showed an increase in the rate of admissions for pain (nontransfused) as subjects aged (r2=0.19, p<0.0001). Thus, the significant improvement in admission rate for pain during transfusion, while the child continues to age, further accentuates the impact of transfusion on the natural history of pain in SCD. In summary, our data suggest that chronic transfusion reduces hospital admissions for pain and acute chest syndrome in children with SCD. Our data also support the notion that chronic transfusion is an effective treatment to prevent not only stroke, but also other painful, life-threatening and life-limiting complications of sickle cell disease. Disclosures No relevant conflicts of interest to declare.

scholarly journals Classification and Evolution of Patterns of Erythropoiesis in Polycythemia Vera as Studied by Iron Kinetics

Blood ◽  
1966 ◽  
Vol 28 (6) ◽  
pp. 807-829 ◽  
Author(s):  
M. POLLYCOVE ◽  
H. S. WINCHELL ◽  
J. H. LAWRENCE ◽  
N. Kusubov
Keyword(s):  
Cell Survival ◽  
Polycythemia Vera ◽  
Selective Advantage ◽  
Red Cell ◽  
Class Iii ◽  
Total Blood ◽  
Myeloid Metaplasia ◽  
Splenic Sequestration ◽  
Red Cell Survival ◽  
Cell Lifespan

Abstract 1. Patients with polycythemia vera may be classified according to their erythropoietic pattern. Erythropoiesis is abnormally increased in all classes. Class I is characterized by normal red cell lifespan. Class II is characterized by shortened red cell lifespan; in Class IIa the shortened red cell survival is related to splenic sequestration of RBC; in Class IIb the markedly shortened red cell survival is predominantly related to intramedullary hemolysis. Class III is characterized by extramedullary erythropoiesis. Patients in Classes I and IIa are in relatively earlier phases of their disease and frequently are found to develop red cell kinetics of Class III as their disease progresses. Conversely, patients in Classes IIb and III are generally late in the course of their disease and have previous hematologic findings that suggest that they originally had the red cell kinetic patterns of Classes I and IIa. 2. As the duration of their disease increases, patients with polycythemia vera generally have a progressive shortening of red cell lifespan which is incompletely compensated by a progressive decrease in circulating red cell volume. However, total blood volume remains elevated since the plasma volume increases. These changes occur whether or not the patient receives radiation therapy. Similar changes may occur in white cell and platelet production and functional survival. It is suggested that the natural history of the disease may be characterized by progressive emergence of hematopoietic cell clones which have a selective advantage for reproduction associated with altered functional survival. 3. The results suggest the potential usefulness of iron, and occasionally of splenectomy, in selected polycythemic patients with myeloid metaplasia (Class III) and anemia, dependent upon the presence of the frequent finding of iron deficiency or the occasional finding of splenic sequestration of red cells in excess of splenic erythropoiesis.

Ceftriaxone-induced drug reaction mimicking acute splenic sequestration crisis in a child with hemoglobin SC disease

Transfusion ◽  
2018 ◽  
Vol 58 (4) ◽  
pp. 879-883 ◽  
Author(s):  
Nancy L. Van Buren ◽  
Jed B. Gorlin ◽  
Robyn C. Reed ◽  
Jerome L. Gottschall ◽  
Stephen C. Nelson
Keyword(s):  
Drug Reaction ◽  
Splenic Sequestration ◽  
Hemoglobin Sc Disease
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