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GATA2 deficiency is characterized by monocytopenia, deficiency of dendritic cells, and a variable degree of lymphocytopenia affecting B cells and NK cells, leading to an enhanced risk of mycobacterial, viral, and fungal infections. Here we present a patient with a heterozygous intronic GATA2 mutation who acquired a fatal disseminated mycosis due to the black yeast-like fungus Arthrocladium fulminans following an infection with Mycobacterium sherrisii. This case illustrates that in patients with severe uncommon infections, immunodeficiency syndromes must be ruled out.
This article was published in the following journal.
Name: Respiration; international review of thoracic diseases
The transcription factor GATA2 is an anti-adipogenic factor whose expression is downregulated during adipocyte differentiation. The present study attempted to clarify the molecular mechanism underlyin...
Preeclampsia is a pregnancy complication associated with elevated placental secretion of anti-angiogenic factors, maternal endothelial dysfunction and organ injury. GATA2 is a transcription factor exp...
Could forms of communication between therapists and inpatients distinguish (un-) similarities of different jeopardizing situations in psychiatry? Explorative cluster- and discriminance analysis of dat...
•Active cancers and inflammation increase the risk for venous thromboembolism. It is unknown whether this applies to mycosis fungoides and parapsoriasis.•Patients with mycosis fungoides and paraps...
Histoplasmosis usually presents primarily as lung infection. Occasionally, mainly in immunocompromised hosts, it can spread and cause systemic manifestations. Skin lesions have been reported in 10 to ...
Purpura fulminans (PF) is a rare life-threatening infectious disease characterized by the association of a sudden and extensive purpura together with acute circulatory failure. The mortali...
The purpose of the study is to develop a prognostic index model for the rare disease of mycosis fungoides and sezary syndrome. This will be done by collecting standardized clinical data at...
RATIONALE: The presence of specific serum proteins may allow a doctor to determine if a patient has mycosis fungoides/cutaneous T-cell lymphoma. PURPOSE: This clinical trial is studying h...
This study evaluates the effectiveness — as judged by complete response — of a single four-day treatment with the fusion protein A-dmDT390-bisFv(UCHT1) compared to oral Zolinza (Vorino...
The purpose of this study is to determine if combination of PUVA with interferon alpha is better than PUVA alone to treat mycosis fungoides stage Ia Ib or IIa.
A severe, rapidly fatal reaction occurring most commonly in children following an infectious illness. It is characterized by large, rapidly spreading skin hemorrhages, fever, or shock. Purpura fulminans often accompanies or is triggered by DISSEMINATED INTRAVASCULAR COAGULATION.
A disorder characterized by procoagulant substances entering the general circulation causing a systemic thrombotic process. The activation of the clotting mechanism may arise from any of a number of disorders. A majority of the patients manifest skin lesions, sometimes leading to PURPURA FULMINANS.
An essential GATA transcription factor that is expressed primarily in HEMATOPOIETIC STEM CELLS.
The right of the patient or the patient's representative to make decisions with regard to the patient's dying.
A mitosporic Onygenaceae fungal genus which causes adiaspiromycosis, a pulmonary mycosis of man and rodents. One of its teleomorphs is Ajellomyces.
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