Sickle Cell Disease

The ribs are sclerotic but "lace-like," secondary to marrow hyperplasia.
The proximal humeri are sclerotic and deformed.
- The radiographic appearance of the humeral heads is one of avascular necrosis (AVN).
The vertebral bodies (always challenging to evaluate on chest radiographs) have an "log cabin" or "H" shape.
- This can be appreciated on both AP as well as Lateralviews

Other findings which might have been seen on this film but, in this case were not, include:

Prominent cardiomegaly and failure (CHF).
Infiltrates secondary to sepsis.
Prominence of interstitial markings (secondary to infarction).
Calcified gallstones (calcium bilirubinate) or RUQ surgical clips s/p surgery for the same.
Splenic calcification (auto-infarction).
Osteomyelitis or septic arthritis.
Fracture(s).
Joint effusions (usually knee, elbow).

You may wish to review some facts concerning Sickle Cell Disease:

Abnormal red cells (RBC's) in which glutamic acid has replaced valine in the beta chain of hemoglobin.
Deformity of RBC leads to vaso-occlusion and tissue death secondary to ischemia.
- Clinically, "painful crises" ensue.
- The main differential diagnosis is infarction versus osteomyelitis (Staphlacoccus, Salmonella common).
  - Bone scan and MR scan may be helpful in distinguishing.
Marrow hyperplasia leads to increased lucency in bone while infarction, and possible myelofibrosis, lead to osteosclerosis.
"Log cabin" or "H" shaped vertebral bodies are highly suggestive of Sickle Cell Disease, and related disorders.
- May be secondary to epiphyseal infarction or epiphyseal growth disturbance or ,perhaps, a combination of the two.
- Smoothly indented, or "fish-mouth" vertebrae can be seen in Sickle Cell Disease but are not characteristic of it.
The reader is referred to the appropriate radiographic literature for discussion of renal papillary necrosis (kidneys) and other topics not covered here.

Ref: Resnick, D., Bone and Joint Imaging, W.B. Saunders, Philadelphia, PA, 1989, pp. 662-668.