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FA Q&A 3rd edition states that the most common finding in Hyper IgM syndrome is neutropenia, with normal no. of lymphocytes. Anyone know why?
Why neutropenia in Hyper IgM syndrome?
- Thread starter goal270
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Hyper IgM Syndromes | Immune Deficiency Foundation
Approximately half of the patients with XHIGM or CD40 deficiency develop neutropenia (low count of granulocyte white blood cells), either transiently or persistently. The cause of the neutropenia is unknown, although most patients respond to treatment with colony stimulating factor, G-CSF. Severe neutropenia is often associated with oral ulcers, proctitis (inflammation and ulceration of the rectum) and skin infections.
Autoimmune disorders may also occur in patients with XHIGM syndrome or CD40 defects. Their manifestations may include chronic arthritis, low platelet counts (thrombocytopenia), hemolytic anemia, hypothyroidism and kidney disease.
Approximately half of the patients with XHIGM or CD40 deficiency develop neutropenia (low count of granulocyte white blood cells), either transiently or persistently. The cause of the neutropenia is unknown, although most patients respond to treatment with colony stimulating factor, G-CSF. Severe neutropenia is often associated with oral ulcers, proctitis (inflammation and ulceration of the rectum) and skin infections.
Autoimmune disorders may also occur in patients with XHIGM syndrome or CD40 defects. Their manifestations may include chronic arthritis, low platelet counts (thrombocytopenia), hemolytic anemia, hypothyroidism and kidney disease.
