scholarly journals Functional asplenia in hemoglobin SC disease

Blood ◽  
1995 ◽  
Vol 85 (8) ◽  
pp. 2238-2244 ◽  
Author(s):  
PA Lane ◽  
JL O'Connell ◽  
JL Lear ◽  
ZR Rogers ◽  
GM Woods ◽  
...  
Keyword(s):  
Blood Cells ◽  
Prospective Multicenter Study ◽  
Reliable Measure ◽  
Sickle Hemoglobin ◽  
Splenic Function ◽  
Hemoglobin C ◽  
Routine Administration ◽  
Life Threatening ◽  
Infants And Young Children ◽  
Hemoglobin Sc Disease

The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life-threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20% were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20% were found in subjects with preserved splenic function. Pit counts greater than 20% were present in 0 of 59 subjects (0%) less than 4 years of age, in 19 of 86 subjects (22%) 4 to 12 years of age, and in 25 of 56 subjects (45%) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7% +/- 9.5%) than splenectomized subjects without hemoglobinopathy (38.5% +/- 8.8%) or with sickle cell anemia (20.5% +/- 1.9%; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3% and 41.7% died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (> 20% in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.

Oral Penicillin Prophylaxis in Children with Impaired Splenic Function: A Study of Compliance

PEDIATRICS ◽  
1982 ◽  
Vol 70 (6) ◽  
pp. 926-930
Author(s):  
George R. Buchanan ◽  
Jane D. Siegel ◽  
Susan J. Smith ◽  
Bonnie M. DePasse
Keyword(s):  
Poor Compliance ◽  
Disc Diffusion ◽  
Cell Disease ◽  
Splenic Function ◽  
Oral Penicillin ◽  
Diffusion Technique ◽  
Life Threatening ◽  
Penicillin Prophylaxis ◽  
Urine Specimens ◽  
Infants And Young Children

One measure used to prevent overwhelming sepsis due to Streptococcus pneumoniae in children with defective splenic function is oral penicillin prophylaxis. However, a frequently cited argument against this approach is the likelihood of poor compliance. Compliance was studied by examining urine specimens for penicillin by the Sarcina lutea disc diffusion technique in 22 surgically asplenic children, two patients following bone marrow transplantation, and 38 infants and young children with sickle cell disease. Multiple specimens (mean 3.5 per patient) were examined in 43 of the children. Overall, 125/188 (66%) of the urine samples contained penicillin, indicating compliance within the previous 12 to 24 hours. Compliance tended to improve on subsequent clinic visits. These relatively good results were attributed to an intensive educational program in which repetitive efforts are made to counsel patients and parents about the risks of life-threatening infection. Poor compliance should no longer be invoked as a reason not to study the efficacy of prophylactic penicillin in functionally or surgically asplenic subjects.

scholarly journals Revisiting Spleen Function and Pneumococcal Risk in Children with Hemoglobin SC Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 768-768
Author(s):  
Charlotte Pourdieu ◽  
Sara El Hoss ◽  
Enora Le Roux ◽  
Justine Pages ◽  
Berengere Koehl ◽  
...  
Keyword(s):  
Age Groups ◽  
Pneumococcal Infection ◽  
Cross Sectional Study ◽  
Control Group ◽  
Healthy Children ◽  
Cross Sectional ◽  
Splenic Function ◽  
Routine Administration ◽  
Adolescents And Adults ◽  
Hemoglobin Sc Disease

Abstract Spleen dysfunction and susceptibility to pneumococcal infection is a well known feature in homozygous sickle cell disease (HbSS), whilst to date splenic function in hemoglobin SC disease (HbSC) has been poorly investigated. The aim of this study was to analyze spleen function in children with HbSC disease using a high-throughput validated method (1) and to examine if the current recommendations regarding pneumococcal risk are appropriate in this population. Spleen function was evaluated using a flow cytometry quantification of red blood cells (RBCs) with Howell-Jolly bodies (HJBs), in a cross-sectional study of patients at steady state during an outpatient visit in an expert center. Quantification of HJB-RBCs was performed in children with HbSC disease aged &lt; 10 years and compared to children with HbSS disease or healthy children of the same age groups, or splenectomized children. Additional exploratory analysis was performed according to age (under or above the age of 5 years old) and treatment group (hydroxyurea). The median (Q1-Q3) HJB-RBCs count was 16 (11-28.25) /100.000 RBCs in 40 HbSC children (Figure 1). This result was not statistically different from the control group of 22 healthy children (p=0.96) nor in subgroups &lt; or ≥ 5 years old, indicating that children with HbSC under 10 years have a preserved splenic function. Expectedly, the HJB-RBCs counts differed significantly from splenectomized children (419 (296-489)/100.000 RBCs, n=15, p&lt;0.0001). By contrast, among the 53 HbSS children, the median HJB-RBCs count was 134 (29-216) /100.000 RBCs, differing significantly from HbSC children (p&lt;0.0001). In HbSS children, HJB-RBCs counts increased significantly with age (r=0.30, p=0.03), showing important variability among subjects but did not reach the level found in splenectomized patients suggesting that complete loss of spleen function occurs presumably later in a majority of children in this population. Treatment with hydroxyurea did not significantly impact HJB-RBCs counts in a subgroup analysis in HbSS children. The result of this study suggests that spleen function in children under 10 years old with HbSC is not altered. The routine administration of prophylactic penicillin to young children with SC disease may therefore be questioned. Similarly, fever in children with HbSC under 3 years old may not require parenteral antibiotics as it is generally currently recommended by analogy to children with HbSS. Functional or anatomical asplenia in children with HbSC is delayed compared to those with HbSS at least after the first decade of life. Future large cohort studies using similar methodology will allow better evaluation of the pneumococcal risk in adolescents and adults with Hb SC disease. Bibliography (1) El Hoss S, Dussiot M, Renaud O, Brousse V, El Nemer W. A novel non-invasive method to measure splenic filtration function in humans. Haematologica. oct 2018;103(10):e436-9. Figure 1 Figure 1. Disclosures El Nemer: Hemanext: Consultancy.

Hemoglobin crystals of the red blood cells in the human renal cortex: electron microscopic observations of the renal lithiasis

1978 ◽  
Vol 36 (2) ◽  
pp. 480-481
Author(s):  
Kosuke Ueda ◽  
Hiroto Washida ◽  
Nakazo Watari
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Renal Cortex ◽  
Uranyl Acetate ◽  
Osmic Acid ◽  
Renal Lithiasis ◽  
Electron Microscopic ◽  
Hemoglobin C ◽  
First Case ◽  
Ultrathin Sections

IntroductionHemoglobin crystals in the red blood cells were electronmicroscopically reported by Fawcett in the cat myocardium. In the human, Lessin revealed crystal-containing cells in the periphral blood of hemoglobin C disease patients. We found the hemoglobin crystals and its agglutination in the erythrocytes in the renal cortex of the human renal lithiasis, and these patients had no hematological abnormalities or other diseases out of the renal lithiasis. Hemoglobin crystals in the human erythrocytes were confirmed to be the first case in the kidney.Material and MethodsTen cases of the human renal biopsies were performed on the operations of the seven pyelolithotomies and three ureterolithotomies. The each specimens were primarily fixed in cacodylate buffered 3. 0% glutaraldehyde and post fixed in osmic acid, dehydrated in graded concentrations of ethanol, and then embedded in Epon 812. Ultrathin sections, cut on LKB microtome, were doubly stained with uranyl acetate and lead citrate.

Bacterial Infection and Splenic Reticuloendothelial Function in Children with Hemoglobin SC Disease

PEDIATRICS ◽  
1983 ◽  
Vol 72 (1) ◽  
pp. 93-98
Author(s):  
George R. Buchanan ◽  
Susan J. Smith ◽  
Christine A. Holtkamp ◽  
John P. Fuseler
Keyword(s):  
Bacterial Infection ◽  
Bacterial Infections ◽  
Infection Risk ◽  
Bacterial Disease ◽  
Normal Children ◽  
Splenic Function ◽  
Focus Of Infection ◽  
Serious Bacterial Infections ◽  
Primary Focus ◽  
Hemoglobin Sc Disease

Although the epidemiology and pathophysiology of serious bacterial infection in homozygous sickle cell anemia (SS disease) have become increasingly well understood, information about infection risk and splenic reticuloendothelial function in hemoglobin SC disease is quite limited. Therefore, the type and frequency of invasive bacterial disease were examined in 51 children with SC disease followed for 370 person-years and splenic function was assessed in 31 patients by quantitation of pitted erythrocytes. Seven serious bacterial infections occurred in four of the patients, five due to Streptococcus pneumoniae and two to Haemophilus influenzae. A primary focus of infection was present in all episodes, none of which proved fatal. Although 30 episodes of pneumonia or chest syndrome occurred in 20 of the patients, a bacterial etiology was proven in only three instances. Splenic function was usually impaired, with a mean pit count of 7.1% ± 8.2% (range 0% to 22.9%). This is significantly greater than normal, but less than pit counts in patients with SS disease or asplenic subjects. Children with SC disease may have a greater risk of bacterial infection than normal children, but their infection rate is not nearly as high as that in patients with SS disease.

NORMAL GRANULOCYTE INFUSION THERAPY FOR ASPERGILLOSIS IN CHRONIC GRANULOMATOUS DISEASE

PEDIATRICS ◽  
1973 ◽  
Vol 51 (2) ◽  
pp. 230-233
Author(s):  
Andrew A. Raubitschek ◽  
Alan S. Levin ◽  
Daniel P. Stites ◽  
Edward B. Shaw ◽  
H. Hugh Fudenberg
Keyword(s):  
Adverse Effects ◽  
Chronic Granulomatous Disease ◽  
Blood Cells ◽  
White Blood Cells ◽  
Infusion Therapy ◽  
Granulomatous Disease ◽  
Transient Improvement ◽  
Life Threatening ◽  
Granulocyte Function

An 8-year-old boy with chronic granulomatous disease (CGD) was admitted in moribund condition with aspergillus pneumonia. Because of the gravity of the situation, normal granulocyte infusions were used as adjuncts to the more conventional antimicrobial therapy. White blood cells, derived from a total of 58 units of whole blood obtained by leukophoresis of the father, were given in two separate doses. The first dose, totaling 2.8 x 1010 granulocytes, was coincident with significant improvement, and the second, totaling 3.0 x 1010 granulocytes, was coincident with the onset of clinical improvement and interim recovery. Transient improvement in in vitro granulocyte function was noted in cells taken from the patient's blood immediately after infusion. No adverse effects of the infusions were noted in either the patient or the donor. Although it is impossible to divorce the therapeutic effect of the granulocyte infusions from the more conventional therapy, we conclude that normal granulocyte infusions can be considered a valid adjunct in children with CGD who are suffering from a life-threatening infection.

Life-threatening thrombotic thrombocytopenic purpura (TTP) in a patient with sickle cell-hemoglobin C disease

2003 ◽  
Vol 82 (11) ◽  
pp. 702-704 ◽  
Author(s):  
H. E. Lee ◽  
V. J. Marder ◽  
L. J. Logan ◽  
S. Friedman ◽  
B. J. Miller
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Sickle Cell ◽  
Thrombocytopenic Purpura ◽  
Hemoglobin C ◽  
Life Threatening

scholarly journals Influence of Haptoglobin Polymorphism on Stroke in Sickle Cell Disease Patients

Genes ◽  
2022 ◽  
Vol 13 (1) ◽  
pp. 144
Author(s):  
Olivia Edwards ◽  
Alicia Burris ◽  
Josh Lua ◽  
Diana J. Wilkie ◽  
Miriam O. Ezenwa ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Cells ◽  
Cell Disease ◽  
Free Hemoglobin ◽  
Cerebral Tissue ◽  
Sickle Hemoglobin ◽  
Haptoglobin Polymorphism

This review outlines the current clinical research investigating how the haptoglobin (Hp) genetic polymorphism and stroke occurrence are implicated in sickle cell disease (SCD) pathophysiology. Hp is a blood serum glycoprotein responsible for binding and removing toxic free hemoglobin from the vasculature. The role of Hp in patients with SCD is critical in combating blood toxicity, inflammation, oxidative stress, and even stroke. Ischemic stroke occurs when a blocked vessel decreases oxygen delivery in the blood to cerebral tissue and is commonly associated with SCD. Due to the malformed red blood cells of sickle hemoglobin S, blockage of blood flow is much more prevalent in patients with SCD. This review is the first to evaluate the role of the Hp polymorphism in the incidence of stroke in patients with SCD. Overall, the data compiled in this review suggest that further studies should be conducted to reveal and evaluate potential clinical advancements for gene therapy and Hp infusions.

Nonmalignant Disorders of Leukocytes

2011 ◽  
Author(s):  
Alison M. Schram ◽  
Nancy Berliner
Keyword(s):  
Blood Cells ◽  
Langerhans Cell Histiocytosis ◽  
White Blood Cells ◽  
Cell Types ◽  
Immune Defense ◽  
Cancer Surveillance ◽  
Vasoactive Substances ◽  
Clinical Disorders ◽  
Life Threatening ◽  
And Function

Leukocytes, also known as white blood cells, are hematologic cells important for a host’s immune defense. They comprise several diverse cell types including lymphocytes, neutrophils, monocytes, macrophages, and eosinophils. Each plays a unique and important role in fighting infection, cancer surveillance, and maintaining immune homeostasis. Leukocytes exert their effect and interact with host and foreign cells through the release of cytokines, chemokines, enzymes, and vasoactive substances. Altered number and function of these cells can lead to clinical disorders that range from benign to severe and life-threatening. Here we review the diagnosis, natural history, and treatment of nonmalignant disorders of leukocytes. This review contains 100 references, 6 figures, and 9 tables. Key Words: eosinophilia, hemophagocytic histiocytosis, Langerhans cell histiocytosis, lymphocytopenia, lymphocytosis mastocytosis, neutropenia, neutrophilia

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