Wednesday, November 23, 2011

anecdotes about non-hemic autoimmunity

Unusual Autoimmune Complications in Chronic Lymphocytic Leukemia
Moonjung Jung1, Lawrence Rice1
The Methodist Hospital, Weill Cornell Medical College, Houston, TX

1. Myasthemia Gravis (MG)

A 56-year-old man was recently diagnosed with MG. His muscle weakness and difficulty swallowing had improved only moderately with weekly plasmapheresis, azathioprine and mestinon. Following a diarrheal illness treated with ciprofloxacin, he was admitted to the ICU for respiratory failure from respiratory muscle weakness. Hemoglobin rapidly fell to 6.7 g/dL. His peripheral blood smear, flow cytometry and Coombs' tests revealed CLL and autoimmune hemolytic anemia. After chemotherapy, his blood counts normalized and his symptoms of MG resolved; he remains in clinical remission of CLL and MG off therapy.

2. Von Willebrand's Disease (vWD)

A 48-year-old man presented with severe headache and was found to have a spontaneous subdural hematoma by CT scan. He had experienced weight loss, easy bruising and epistaxis recently. There was lymphadenopathy on exam. Blood counts, flow cytometry, and coagulation tests revealed CLL and type IIA vWD with a circulating vWF inhibitor. After chemotherapy, the CLL remitted and coagulation studies normalized. With relapse of CLL four years later, the bleeding diathesis re-emerged

3. Aquired Angioedema

Three patients with known stable CLL for years, presented with episodes of severe abdominal cramping, nausea and vomiting. This led to numerous Emergency Room visits, hospital admissions, invasive diagnostic tests, and disability. A deficiency of C1 esterase inhibitor was discovered in all cases. With danazol and chemotherapy for CLL, the patients had response of CLL, normalization of C1 esterase inhibitor, and no recurrence of abdominal pains.

In some of our patients, signs and symptoms of CLL were neglected at the onset of the autoimmune problem. Earlier consideration of underlying CLL would have facilitated earlier effective treatment. In all our patients, the activity of the autoimmune complications closely correlated with the activity of CLL. Immune dysregulation characterizes CLL, and effective treatment offers the possibility of reversing the environment ripe for autoimmunity.

These are more anecdotes. The plural of anecdote is not evidence. I would dismiss these cases as coincidence.

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