Rare disease pits white blood cells against organs

By

Community

October 30, 2018 - 9:14 AM

Dear Dr. Roach: I am an 83-year-old male and have been in excellent health. My vital signs all are normal, my heart function is normal, MRI of my brain and a full-body X-ray of my bones are normal, but a CT scan of my abdomen showed organs with “fuzzy” material around the exterior. I recently was diagnosed with Langerhans cell histiocytosis. The symptoms are: a severe, very itchy upper torso body rash on both front and back; swollen feet, ankles and lower legs; and a general feeling of unstable tiredness. I am not able to sleep well because of the itchiness.

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.

Answer: 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. The liver and spleen are organs that, if affected, are associated with a worse prognosis.

Related
July 18, 2019
February 21, 2019
December 31, 2018
November 5, 2018