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mastocytosis/fever

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Mast cell disorders are defined by an abnormal accumulation of tissue mast cells in one or more organ systems. In systemic mastocytosis, at least one extracutaneous organ is involved by definition. Although, systemic mastocytosis usually represents with skin lesion called urticaria pigmentosa, in a

Fever of unknown origin as unique symptom of an indolent mastocytosis.

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A 39-years-old man afferred to our hospital for a fever lasting for more than 6 months, without abnormalities at physical examination (in particular no skin alterations); a recent laboratory and instrumental investigation was ineffective and so a fever of unknown origin (FUO) was diagnosed Since he

[Systemic mastocytosis and fever of unknown origin].

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BACKGROUND Laparoscopic splenectomy has become the surgical procedure of choice for various diseases of the spleen. Portal vein thrombosis (PVT) after splenectomy occurs in 0.5% to 22% of patients. Symptoms are nonspecific and include fever, abdominal pain, and epigastric distress. Risk factors for
We report a male infant with onset of an extensive bullous eruption at the age of 45 days. Staphylococcal scalded skin syndrome (SSSS) was suspected. Bullous mastocytosis was diagnosed by cytodiagnosis and confirmed by histologic examination. Three serious relapses were noted in a 2-year follow-up,
BACKGROUND Fever of unknown origin (FUO) still remains a diagnostic challenge, while diagnosis may remain obscure for several weeks or months. The role of tissue biopsy is crucial in the diagnostic approach. We report a series of 130 consecutive patients with FUO who had undergone a bone marrow

[A case of aggressive systemic mastocytosis].

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The study presents a case of systemic mastocytosis, discovered in 64 years old male, after two years of persistent fever and chest pain with dyspnea. The authors describe differential diagnostics including imaging and laboratory procedures. Presented material contains pictures of histopathological

Shock in an infant with bullous mastocytosis.

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A 6-month-old infant had bullous lesions on his posterior neck, upper trunk, and extremities for two months prior to admission for fever and shock. He had an elevated white blood cell count with left shift and normal platelet count, but abnormal coagulation studies. He was treated with intravenous
BACKGROUND Mastocytosis is an abnormal proliferation of mast cells and their subsequent accumulation in various organs. Diagnosis of mast cell disease relies on proper identification of abnormal mast cells. METHODS A 55-year-old man presented with a history of fever for several months, associated
Systemic mast cell disease (SMCD) is a rare disease often associated with symptoms of general malaise, pruritus, diarrhea, vomiting, fever, urticaria pigmentosa, hepatosplenomegaly and lymphadenopathy. We reported a case of SMCD associated with cutaneous xanthoma and serum hyper IgE. Skin biopsy

Systemic mastocytosis with review of gastrointestinal manifestations.

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A 63-year-old man presented with fever, splenomegaly, steatorrhea, diarrhea, and weight loss. A tissue diagnosis of systemic mastocytosis was made. This case is unusual in that diarrhea and steatorrhea were present in the absence of skin lesions and because fever was a prominent symptom. Thus,
Radiation alone can be used to treat mast cell tumors that are not likely to metastasize (that is, differentiated or localized lymphorecticular tumors). In patients with mast cell tumors that are likely to metastasize (that is, undifferentiated, metastatic mast cell disease or generalized

Response to cladribine in patient with systemic mastocytosis.

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BACKGROUND Systemic mastocytosis is a heterogeneous group of hematological disorders characterized by accumulation of mast cells in different organs. METHODS A 41-year-old woman presented with a three-year history of fatigue, occasional diarrhea, mild fever, skin rash and splenomegaly. Laboratory

Aggressive systemic mastocytosis mimicking sclerosing cholangitis.

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A 43 year-old woman presented with fever, abdominal pain, epato-splenomegaly, ascites, cholestasis, anemia, thrombocytopenia and previous diagnosis of sclerosing cholangitis based on liver biopsy and endoscopic retrograde cholangiopancreatography(ERCP). The bone marrow biopsy and the revision of
The term mastocytosis denotes a heterogeneous group of rare hematological disorders characterized by abnormal accumulation of mast cells. While cutaneous mastocytosis is relatively frequent mast cell leukemia belongs to the rarest forms of human leukemia. In the following we present the case of an
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