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902 PART 7: Hematologic and Oncologic Disorders

Response rate to immunosuppressive agent such as glucocorticoids,
rituximab, and vincristine has been discouraging as well. Protein A • Serum creatinine levels of 1 to 1.5 mg/dL often indicate significant
immunoadsorption has been used with some success (response rates renal dysfunction.
45%-75%) by removing immunoglobulin G and circulating immune • The most common intensive care management problems in
complexes from the serum with significantly fewer adverse effects that patients with sickle cell disease include the acute chest syndrome,
plasma exchange. 98,99 very severe anemia, sepsis, stroke, priapism, splenic sequestration,
or right heart failure associated with acute chest syndrome and/or
acute severe hemolytic anemia.
KEY REFERENCES
• The acute chest syndrome is a form of acute lung injury that occurs
• Freifeld AG, Bow EJ, Sepkowitz KA, et al. Clinical practice guide- in 10% to 20% of patients hospitalized in vaso-occlusive pain crisis,
line for the use of antimicrobial agents in neutropenic patients often caused by fat embolization syndrome or pneumonia.
with cancer: 2010 update by the infectious diseases society of • Secondary pulmonary hypertension, defined by right heart cath-
america. Clin Infect Dis. February 2011;52(4):e56-e93. eterization and often unrecognized, occurs in 10% of adult patients
• Geiger S, Lange V, Suhl P, Heinemann V, Stemmler HJ. Anticancer with sickle cell disease.
therapy induced cardiotoxicity: review of the literature. Anticancer • Red cell transfusion is an important treatment for most patients
Drugs. July 2010;21(6):578-590. with sickle cell disease requiring intensive care management.
• Kintzel PE. Anticancer drug-induced kidney disorders. Drug Saf. • Rapid exchange transfusion is indicated for central nervous system
January 2001;24(1):19-38. events, serious respiratory disease, or multiorgan failure.
• Lee-Chiong T Jr, Matthay RA. Drug-induced pulmonary edema • Transfusion management in patients with sickle cell disease
and acute respiratory distress syndrome. Clin Chest Med. March requires investigation of alloimmunization history.
2004;25(1):95-104. • Preoperative red cell transfusion and detailed supportive care are
• Peterson DE, Bensadoun RJ, Roila F. Management of oral and gas- advisable for significant surgery in patients with sickle cell disease.
trointestinal mucositis: ESMO Clinical Practice Guidelines. Ann
Oncol. May 2010;21(suppl 5):v261-v265.
• Richardson PG, Soiffer RJ, Antin JH, et al. Defibrotide for the Sickle cell disease is a highly prevalent disease in the United States, affecting
treatment of severe hepatic veno-occlusive disease and multi- 1 in 500 African American infants. It is common in individuals of African,
organ failure after stem cell transplantation: a multicenter, ran- Caribbean, Mediterranean, Arab, and other Middle Eastern descent. It is a
domized, dose-finding trial. Biol Blood Marrow Transplant. July genetic disorder with an autosomal recessive inheritance pattern. Sickle cell
2010;16(7):1005-1017. disease is often called “the first molecular disease” because the biochemi-
• Schiff D, Wen PY, van den Bent MJ. Neurological adverse effects cal alteration in sickle hemoglobin described by Linus Pauling in 1948
was one of the first lesions identified at the molecular level for a human
caused by cytotoxic and targeted therapies. Nat Rev Clin Oncol. disease. Sickle hemoglobin forms rod-like polymers in deoxygenated red
October 2009;6(10):596-603. cells in areas of the circulation, with low oxygen tension, acidosis, or hyper-
• Zanotti KM, Markman M. Prevention and management of osmolarity. Sickle hemoglobin polymerization causes a host of secondary
antineoplastic-induced hypersensitivity reactions. Drug Saf. molecular and cellular changes, many of which impair blood flow and con-
2001;24(10):767-779. tribute to tissue damage. The microcirculation can be acutely or chronically
• Zorzi D, Laurent A, Pawlik TM, Lauwers GY, Vauthey JN, Abdalla impaired in virtually any organ in the body, resulting in the characteristic
EK. Chemotherapy-associated hepatotoxicity and surgery for crisis pattern of intermittent pain and acute organ injury superimposed on
colorectal liver metastases. Br J Surg. March 2007;94(3):274-286. the gradual development of chronic organ failure.
• Zupancic M, Shah PC, Shah-Khan F. Gemcitabine-associated Despite the early progress in a molecular understanding of sickle cell
thrombotic thrombocytopenic purpura. Lancet Oncol. July disease, its treatment remained largely palliative for many decades. In
2007;8(7):634-641. recent years, the longevity of patients with sickle cell disease has been
prolonged by the institution of prophylactic penicillin treatment and
immunization to decrease mortality rate from pneumococcal sepsis.
Chronic transfusion therapy for selected patients has improved out-
REFERENCES come, and acute transfusion therapy is the central intervention for most
complications requiring admission to the intensive care unit (ICU).
Complete references available online at www.mhprofessional.com/hall
Hydroxyurea, the first treatment approved by the Food and Drug
Administration for sickle cell disease, decreases disease severity and mor-
tality rate. Most recently, new paradigms have emerged regarding the
1,2
pathophysiology of secondary complications of sickle cell disease, with
CHAPTER Sickle Cell Disease much of this involving the pathological effects of intravascular hemolysis
which impairs normal vascular function, in part by disrupting the nitric
96 Gregory J. Kato oxide (NO) pathway (Fig. 96-1). These new paradigms have triggered
3-5
a wave of research seeking to translate these basic science advances into
Mark T. Gladwin
clinical practice. This chapter reviews general aspects of the genetics and
6
pathophysiology of sickle cell disease, common clinical problems with
an emphasis on those faced in a critical care setting, and contemporary
KEY POINTS therapeutic approaches to these complications of sickle cell disease.
• Sickle cell disease causes a chronic hemolytic anemia associated
with acute and chronic vaso-occlusion. GENERAL BACKGROUND
• Baseline hemodynamic and laboratory values in patients with In 1910, James Herrick first reported the observation of sickle-shaped
sickle cell disease can be confused with sepsis. red cells from the blood of an anemic Chicago dental student from
Grenada. Subsequently, in 1949, Linus Pauling and his colleagues

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