Sickle cell trait in a white Jewish family presenting as splenic infarction at high altitude

Shalev, Oded; Boylen, Alice L.; Levene, C.; Oppenheim, Ariella; Rachmilewitz, Eliezer A.

doi:10.1002/ajh.2830270111

Cited by 17 publications

(10 citation statements)

References 20 publications

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“…Although there is a theoretical risk that G-CSF mobilization may precipitate a crisis in donors with SCT, [3][4][5][6][7] none of the subjects in this study experienced unexpected adverse effects, including symptoms resembling a sickle crisis. White cell counts in our SCT subjects exceeded those recorded for the patients with sickle cell disease who experienced complications from G-CSF administration.…”

Section: Discussionmentioning

confidence: 92%

“…Typically, crises have been precipitated by movement to high altitudes, resulting in pulmonary and splenic infarctions and red cell hemolysis. [3][4][5][6] The use of G-CSF may represent another form of stress. In at least one report, a patient with sickle cell disease given G-CSF for PBSC mobilization developed a pain crisis in association with a white blood cell count of 63.4 ϫ10 9 /L, suggesting that leukocytosis may cause crisis.…”

Section: Introductionmentioning

confidence: 99%

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Mobilization, collection, and processing of peripheral blood stem cells in individuals with sickle cell trait

Kang

Areman

David-Ocampo

et al. 2002

Blood

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Mobilized peripheral blood is increasingly used as the source of hematopoietic stem cells for allogeneic transplantation, currently the only curative approach for sickle cell anemia. However, the safety and feasibility of stem cell mobilization in individuals with sickle cell trait (SCT) has not been documented. This study is a prospective controlled trial to evaluate the safety and feasibility of peripheral blood stem cell (PBSC) mobilization in 8 SCT subjects and 8 control subjects matched for age and race. Mobilization with filgrastim 10 g/kg subcutaneous daily for 5 days was followed by 12-L apheresis on the fifth day. Filgrastim administration was accompanied by similar symptoms in all subjects; no untoward adverse events occurred in either group, including sickle cell crises. CD34 ؉ cell mobilization response was not significantly different between SCT and control subjects. Median CD34 ؉ cell content was also similar in PBSCs collected from SCT versus control subjects, 6.8 versus 3.9 ؋10 6 CD34 ؉ cells/70 kg, P ‫؍‬ .165. Red cell depletion from SCT products was not possible by using hydroxyethyl starch sedimentation but was achievable with ammonium chloride lysis. There was no evidence of gelling of SCT products after thaw, and no difference in cell recovery was seen among red cell-depleted versus nondepleted products. Cryopreservation in 5% dimethyl sulfoxide/6% pentastarch was associated with superior cell recovery (both SCT and control subjects) compared with 10% dimethyl sulfoxide (P ‫؍‬ .001). The study concluded that filgrastim mobilization, large volume apheresis, processing, and cryopreservation appears to be safe in donors with SCT, allowing PBSC use for transplantation in patients with sickle cell anemia. (Blood. 2002;99:850-855)

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Section: Discussionmentioning

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Mobilization, collection, and processing of peripheral blood stem cells in individuals with sickle cell trait

Kang

Areman

David-Ocampo

et al. 2002

Blood

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“…These results could possibly indicate that there is an ab normality in the storage of RBC in spleen among SCT sub jects during exercise. It is known that extreme exertion causes splenic infarction among SCT subjects [10][11][12], Values are mean ±SD (n = 11 for AA and n = 11 for SCT). Mets: The multiple of one metabolic equivalent, where V 0 2 = 3 ml/ min/kg.…”

Section: Discussionmentioning

confidence: 99%

“…Most of these cases have been associated with high ex treme exertion and exposure at high altitudes [1, 2,4 ,6,7]. Nonfatal complications include intravascular coagulation [4,8,9], splenic infarction [10][11][12], rhabdomyolysis and myoglobinuria which may lead to renal failure [4,[8][9][10][11], Values are mean ± SD (n = 11 for AA and n = 11 for SCT). See text for discussion of sta tistical tests.…”

Section: Introductionmentioning

confidence: 99%

Effects of Physical Stress on Complete Blood Count and Venous Blood Gas Profile of Individuals with Sickle Cell Trait

Hardy¹,

Mukherjee

Hinds³

et al. 1992

Acta Haematol

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The association between sickle cell trait (SCT) and adverse effects of exercise has been controversial. While individuals with SCT are at higher risk of sudden death, the mechanism for this outcome remains to be elucidated. In order to shed light on this controversy, we have monitored venous blood count and blood gas parameter values in normal and SCT subjects during treadmill exercise. White and red blood cell counts and hemoglobin changed significantly over time in both the SCT and normal groups, with peak exercise values different from pre-exercise or post-exercise values. Red blood cell counts showed significant group-time interaction; increase in count during exercise was accentuated in SCT subjects. All blood gas parameters showed significant changes over time in both groups. O₂ content was significantly higher in SCT than AA at all time intervals. O₂ saturation, pO₂ and CO binding to hemoglobin showed significant group-time interaction. Furthermore, O₂ saturation for the combined groups was significantly greater at peak exercise and at rest than before exercise. It is possible that treadmill exercise causes microvascular shunting in SCT subjects, leading to a decrease in the peripheral utilization of oxygen.

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“…Higher prevalence of up to 25% has been reported in some populations, but it is very rare to find sickle cell disease or trait in Caucasians [6, 7]. Although considered a benign disorder, it has been associated with numerous complications and adverse events, more so when exposed to conditions that would promote sickling, for example, periods of stress or high altitude [6, 8]. Case reports ranging from pain crisis to rhabdomyolysis, renal failure, and splenic/intestinal infarction have been reported [9, 10].…”

Section: Introductionmentioning

confidence: 99%

Safety of Pegfilgrastim (Neulasta) in Patients with Sickle Cell Trait/Anemia

Kasi

Patnaik

Peethambaram

2013

Case Reports in Hematology

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Pegfilgrastim (Neulasta) is a recombinant filgrastim (human granulocyte colony-stimulating factor (G-CSF)) attached to a polyethylene glycol (PEG) molecule and is given as part of chemotherapy regimens that are associated with significant myelosuppression and risk for febrile neutropenia. Prescribing information available on manufacturer's website for the drug warns us about possible severe sickle cell crises related to the medication but does not report the actual incidence or the use in patients with sickle cell trait. Caution is advised when using it in patients with sickle cell disease. Here we present a case of a Caucasian female with known sickle cell trait (SCT) with no prior complications who developed a presumed sickle cell crisis after getting Neulasta, as a part of the chemotherapy regimen used to treat her breast cancer. Based on our literature review, this appears to be the first case report of a patient with SCT developing a sickle cell crisis with the pegylated form of recombinant filgrastim. Given the dearth of literature regarding the use of G-CSF and its related pegylated forms in patients with sickle cell anemia and sickle cell trait, a discussion of potential mechanisms and review of current literature and guidelines is also presented.

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Sickle cell trait in a white Jewish family presenting as splenic infarction at high altitude

Cited by 17 publications

References 20 publications

Mobilization, collection, and processing of peripheral blood stem cells in individuals with sickle cell trait

Mobilization, collection, and processing of peripheral blood stem cells in individuals with sickle cell trait

Effects of Physical Stress on Complete Blood Count and Venous Blood Gas Profile of Individuals with Sickle Cell Trait

Safety of Pegfilgrastim (Neulasta) in Patients with Sickle Cell Trait/Anemia

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