Disease pits blood cells against organs

Dear Dr. Roach: I am an 83-year-old male and have been in excellent health. My vital signs are normal, my heart function is normal, MRI of my brain and a fullbody X-ray of my bones are normal, but a CT scan of my abdomen showed organs with “fuzzy” material around the exterior. I was diagnosed with Langerhans cell histiocytosis.

My local hematologist has seen only two cases in his 30-year career, both in young children. I consulted with a leading hematologist in a university medical school, and she told me that she has seen five cases this year — it’s apparently very rare, especially in older adults. The recommended treatment is 500 mg capsules once a day of hydroxyurea (Hydrea). After a week of this drug I have felt no results. — J.J.

Langerhans cell histiocytosis is a rare disease, especially in adults (about one in a million). It is now considered a cancer, as the abnormal cell, the histiocyte (a type of white blood cell) infiltrates multiple organs and causes damage to healthy tissues. The skin is a common organ to be affected, and an itchy rash (which can look like just about anything) also is common. It may be the case that you have had this condition far longer than you thought. Other organs can be affected, and I suspect that the abnormal CT scan may represent LCH in the abdomen.

Hydroxyurea often is effective as treatment for LCH. One week is not nearly enough time to see whether the treatment is effective. It may take months for a good response. If indeed your internal organs are affected, more intensive therapy may be recommended.

Getting consultation from an expert is a very good idea for this rare disease. Perhaps the greatest expert in the U.S. for this condition is at Baylor College of Medicine in Houston.