La Revue de médecine interne
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Review Case Reports
[Primary granulocytic sarcoma of the pancreas: efficacy of early treatment with intensive chemotherapy].
In the absence of systemic chemotherapy after the diagnosis of primary granulocytic sarcoma (PGS), all patients will subsequently develop acute myelogenous leukemia (AML). The authors describe a case of PGS of the head of the pancreas found at laparotomy in a 32-year-old man. The patient received early after surgery, two courses of high-dose chemotherapy and, with a follow-up of 2 years, never developed AML. This rare observation illustrates the necessity of further early systemic chemotherapy after surgical excision of PGS.
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Fourty cases of Henoch-Schönlein purpura in adults (21 females, 19 males--age 39 years) are reviewed [departments of nephrology (28 cases) and internal medicine (12 cases)]. Dermatological manifestations occur in 39 cases and are similar to those seen in children. Gastrointestinal involvement (23 cases) usually takes the form of abdominal pains or diarrhea; gastrointestinal haemorrhages are rare. ⋯ In this review, renal insufficiency, hypertension and young age predict a poor outcome. This severe outcome is probably explained by the fact that most of our patients referred to renal units. Unlike in the children, in which the affection is usually an acute illness, Henoch-Schönlein purpura in the adults seems to be a chronic disease, with prognosis depending on the nephropathy.
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Peripheral neuropathy in primary Sjögren's syndrome is common although often unrecognized in mild forms of this affection. They are characterized by a predominently sensory deficiency and can be divided in several entities, of which the most important are: axonal distal symmetric polyneuropathy, either of sensory or sensory-motor presentation, which are supposed to be consecutive to ischemia induced by the vasculitis of the vasa nervorum; sensory neuronopathy mimicking the paraneoplastic syndromes from a clinical and electrophysiological point of view which are supposed to be linked to neuronal degeneration secondary to a lymphocytic infiltration of the dorsal root and ganglia; trigeminal sensory neuropathy, either alone or associated with one of the previously cited forms. ⋯ Tracking down systematically these peripheral neuropathies in patients affected by Sjögren's syndrome is the best way to recognize them. Alternatively, patients with unexplained neuropathies should be evaluated for the presence of Sjögren's syndrome.