The manifestations of the disease are expressed in the most diverse organs and systems, as detailed below:
MUSCULOSKELETAL
OSTEONECROSIS: also known as aseptic necrosis or avascular necrosis, results from infarction of bone trabeculae; usually occurs in the femoral head, proximal humerus and vertebral bodies (known as H-shaped vertebrae). On radiographs they may appear as lytic or blastic areas.
DACTYLITIS (hand-foot syndrome): edema and periosteal reaction on hands and feet due to ischemia, typically occurs in children.
SUBPERIOSTEAL SPONTANEOUS HEMORRHAGE
MARROW HYPERPLASIA (due to chronic anemia): extramedullary hematopoiesis (liver, spleen and/or hematopoietic masses) and persistence of red bone marrow. This persistente may cause expansion of the bone, manifesting the "hair on end" sign, which is the enlargement of the diploc space.
OSTEOMYELITIS: bone infection involving the medullary cavity, typically bacterial etiology
MUSCLE AND/OR SOFT TISSUE NECROSIS
CRANIAL
CEREBRAL ISCHEMIA: on peripheral areas of the white matter
MOYAMOYA SYNDROME: development of thin collateral vessels secondary to occlusion of the internal carotid artery.
ARTERIAL TORTUOSITIES AND ANEURYSM FORMATION:
CARDIOPULMONARY
ACUTE CHEST SYNDROME: consolidation in the lower lobes. Ischemic etiology, more serious.
RECURRING PNEUMONIA: consolidation of variable location. Infectious etiology.
CHRONIC LUNG DISEASE: sequelae of recurrent ischemia.
DILATED CARDIOMYOPATHY: caused by ischemic sequelae of coronary syndromes.
PULMONARY HYPERTENSION: due to chronic hypoxia and chronic pulmonary thromboembolism
ABDOMINAL
SPLEEN: Autosplenectomy: resulting from progressive infarcts and calcium deposition, results in impaired immune status that makes the patient susceptible to infection. Splenic sequestration syndrome: acute accumulation of blood in the spleen, increasing its volume, associated with hemodynamic shock.
KIDNEYS: enlarged (on initial stages; due to increased plasma flow) and chronic kidney disease (long-term; due to acute papillary necrosis and/or renal vein thrombosis).
LIVER: necrosis and cirrhosis of ischemic etiology, as well as sequestration syndrome.
BILIARY TRACT: pigmented gallstones (can cause cholecystitis, choledocholithiasis and pancreatitis)
INTESTINAL ISCHEMIA: propensity for thrombosis due to blood stasis