We report a case of
anaphylactoid shock occurring immediately after the initiation of second
intravenous administration of high-dose
immunoglobulin (
IVIg) in a patient with
Crow-Fukase syndrome. The patient was a 57-year-old woman, who was admitted to our hospital because of
numbness and
muscle weakness in the four extremities, difficulty in walking, and foot
edema. On admission, her skin was dry and rough, and also showing scattered pigmentation, small
hemangiomas, and
hypertrichosis in both legs. She had distal dominant
muscle weakness, more prominent in her legs, and was not able to walk. Deep tendon reflexes in her four extremities were markedly diminished or absent. She had a glove and stocking type of
paresthesia, severe impairment of vibration, and absence of joint position sensation in her four extremities. On laboratory data, serum
vascular endothelial growth factor (
VEGF) was markedly elevated to 5,184 pg/ml (normal: below 220 pg/ml). Cerebrospinal fluid examination revealed cell counts of 2/microliter and
protein level of 114 mg/dl. Abdominal echo showed marked hepatosplenomegaly. On peripheral nerve conduction study, both motor and sensory conduction velocity were undetectable in her legs. We diagnosed her condition as
Crow-Fukase syndrome, and started
IVIg of polyethyleneglycol-treated
gamma-globulin (PEG-glob) at 400 mg/kg/day for 5 consecutive days for
polyneuropathy. Since the first
IVIg mildly improved
muscle weakness, we tried the second
IVIg of PEG-glob. However, immediately after the initiation of second
IVIg of PEG-glob, she developed hypotention,
dyspnea, cold sweating,
cyanosis, and became lethargic. We immediately stopped
IVIg and started
first-aid treatment with
epinephrine and
corticosteroid for these symptoms. This treatment was successful and the patient fully recovered without any sequelae. Since serum
IgE level remained unchanged and lymphocyte stimulation test (LST) was positive against the same rot number of PEG-glob, we diagnosed these symptoms as
anaphylactoid shock. Based on the results of LST, we speculated that PEG-glob was the causative agent of
anaphylactoid reaction. Anaphylactic or
anaphylactoid reaction as adverse effects of
IVIg is very rare, and to our knowledge, there are only 4 previous reports of anaphylactic or
anaphylactoid reaction caused by
IVIg. Therefore, we speculated that the prominent high level of serum
VEGF in the present patient might play a significant contributory role in the development of
anaphylactoid shock, since the vascular permeability of
VEGF is 50,000 times stronger than that of
histamine. We consider that it is necessary to carefully monitor
IVIg of PEG-glob administration for
polyneuropathy in patients with high level of serum
VEGF, like
Crow-Fukase syndrome.