In sickle cell disease

Walters MC. Novel therapeutic approaches in sickle cell disease. Stem cell transplantation for sickle cell disease How and when to intervene Hematology 2002 22-29. 

Endothelial Cells are Targets for Hydroxy Urea Relevance to the Current Therapeutic Strategy in Sickle Cell Disease... 

Hydroxy urea (HU), a hydroxylated derivative of urea, has long been used in the treatment of various forms of neoplastic disorders such as polycythemia vera, head and neck cancer, chronic granulocytic leukemia, and more recently in sickle cell disease, / thalassemia and HIV infection [1],... 

Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP. Mortality in sickle cell disease. 

Hammerman SI, Kourembanas S, Con-ca TJ, Tucci M, Brauer M, Farber H.W. Endothelin-1 production during the acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med 1997 156 280-285. 

The most common abnormal hemoglobin in the United States is hemoglobin S (HbS). Two genes for HbS result in sickle cell disease (SCD) or sickle cell anemia, which occurs in 0.3% of African Americans. One gene for HbS results in sickle cell trait, which occurs in 8% of African Americans. Hemoglobin C, another abnormality, occurs in 2% to 3% of African Americans. 

A. N Gladwin, M. T., Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease, Nature Med. 8 (2002), p. 1383-1389... 

S. H., Increasing fetal hemoglobin in sickle cell disease comparisons of 5-azacytidine (subcutaneous or oral) with hydroxyurea, Trans. Assoc. Am. Physicians 97 (1984), p. 140-145... 

L3. Lathem, W., and Jensen, W., Plasma hemoglobin-binding capacity in sickle cell disease. Blood 14, 1047 (1959). 

Transplant and allergy also intersect in indirect ways. Acute chest syndrome is a frequent complication in patients with sickle cell disease and has been recently reported to be exacerbated or precipitated by asthma and respiratory allergies. Since SCT can halt this pulmonary destruction process, ongoing investigation at our and other institutions is focused on determining genetic and cytokine pathways and modulators for asthma in sickle cell disease. 

Yaster, M., Kost-Byerly, S., and Maxwell, L.G. (2000) The management of pain in sickle cell disease. Pediatr Clin North Am 47 ... 

Hemoglobinopathies are disorders caused either by production of a structurally abnormal Hb molecule (seen in sickle cell disease), synthesis of insufficient amounts of normal Hb subunits (seen in thalassemias), or, rarely, both. 

Jacob, E., Pain management in sickle cell disease, Pain Manage. Nurs., 2, 121, 2001. 

Figure 2-5. Pathologic changes in sickle cell diseases. Vaso-occlusion is a combination of exposure of phosphatidylserine on the red cell membrane, activation of vascular endothelial cells, activation of leukocytes, and a state of increased coagulability. From Steinberg, 1999. Copyright 1999, Massachusetts Medical Society. All rights reserved.

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