General
=========================================================================

• High sinusoidal blood flow in bone marrow predisposes it to blood stasis, causing regional hypoxia, acidosis, sickling and then thrombosis and infarction
• Other complications are osteomyelitis and avascular necrosis

Diagram
=========================================================================

Pathophysiology

Case reports
=========================================================================

• 8 month old girl with sickle cell disease and Salmonella osteomyelitis (Ned Tijdschr Geneeskd 2004;148:1695)
• 23 year old man with sickle cell crisis associated with hemophagocytic lymphohistiocytosis (Am J Hematol 2004;77:229)
• Man with extensive bone marrow necrosis as presenting manifestation of sickle cell disease in Africa (Med Trop (Mars) 2004;64:179)

Micro description
=========================================================================

• May have increased normoblasts and megaloblastic changes due to folate deficiency
• Increased perivascular fibrosis in small and medium sized vessels (Arch Pathol Lab Med 2004;128:634)
• Occasional aplastic crisis

Micro images
=========================================================================

Fibrosis

Infarction of medullary cavity

Peripheral smear images
=========================================================================

Sickled erythrocytes

Positive stains
=========================================================================

• CD1a, CD1b and CD1c in monocytes (Hum Immunol 2004;65:1370)

Additional references
=========================================================================

• eMedicine-Sickle Cell Anemia

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at [email protected] with any questions (click here for other contact information).