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

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 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

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

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.

scholarly journals Acute Splenic Sequestration Crisis in a 70-Year-Old Patient With Hemoglobin SC Disease

2016 ◽  
Vol 4 (1) ◽  
pp. 232470961663836 ◽  
Author(s):  
John J. Squiers ◽  
Anthony G. Edwards ◽  
Alberto Parra ◽  
Sandra L. Hofmann
Keyword(s):  
Splenic Sequestration ◽  
Hemoglobin Sc Disease

Difficulties in the detection of the culprit drug for an adverse drug reaction in a complicated case

2008 ◽  
Vol 149 (19) ◽  
pp. 883-887 ◽  
Author(s):  
Henriette Kopcsányi ◽  
Julianna Feldmann ◽  
Zsófia Péch ◽  
Ágnes Jurcsik
Keyword(s):  
Adverse Drug Reaction ◽  
Drug Reaction ◽  
Complicated Case ◽  
Erythema Exsudativum Multiforme ◽  
Culprit Drug

Az adverz gyógyszerreakciók különféle klinikai megjelenési formái alapján a kiváltó gyógyszerre nehéz következtetni. A gyanúba vett gyógyszerek elhagyása után fellépő javulás vagy az empirikus tapasztalat, esetleg irodalmi adatok alapján lehet a felelős gyógyszert feltételezni. A gyógyszerérzékenység bizonyítása bonyolult, jól átgondolt orvosi tevékenység útján lehetséges, és azokban az esetekben szükséges, amikor a gyanús gyógyszer más vegyületcsoportba tartozó szerrel nem helyettesíthető, és a betegnek feltétlenül szükséges szednie. A jelen közlemény célja egy eset kapcsán körvonalazni azokat a lehetőségeket és veszélyeket, melyek a kivizsgálás során alkalmazhatók, illetve megjelenhetnek. A kivizsgálás során használt módszerek a gyanúba vett gyógyszerekkel – lamotrigin, risperidon, sertralin – zopiclonum, atorvastatin elvégzett epicutan teszt során egyedül a sertralin adott a multiform tünetekkel azonos pozitív reakciót. A pozitív bőrteszt területéről készült hisztológiai vizsgálat eredménye visszautalt az eredetileg észlelt klinikai formára (erythema exsudativum multiforme Stevens–Johnson-szindróma). A beteg bipoláris affektív zavara miatt a negatív bőrtesztet adó készítményekkel per os provokáció történt, ez azonban a beteg számára kockázatos, de az egyetlen megbízható és biztos módszer. Az elvégzett per os expozíció során a törzsön a lamotrigintől már tört adag bevételét követően három órán belül diffúz erythema, 12 órán belül az alkalmazott szteroid- és antihisztamin-kezelés ellenére scarlatiniform exanthema jelent meg. Az eset érdekessége, hogy a gyanúba vett gyógyszerek során epicutan teszttel egy gyógyszer érzékenysége igazolódott, azonban a másik gyanúba vett készítménnyel végzett pozitív per os expozíciós próba is váratlanul tünetet provokált (scarlatiniform, azonnali reakciót). Megállapítható, hogy csupán egy bizonyos módszer még a klinikai képpel való egyező eredmény alapján sem elegendő adverz gyógyszerreakció provokáló tényezőjének bizonyítására.

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