Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Large granular lymphocytic leukemia refers to a clonal expansion of lymphocytes that have abundant cytoplasm and azurophilic granules. The disease is characterized clinically by chronic neutropenia and it may be associated with recurrent pyogenic infections. Except for these infections, cutaneous manifestations of this disease have not been well characterized. We describe a patient with large granular lymphocytic leukemia, which was confirmed by molecular genetics studies, who had a pyoderma gangrenosum-like ulcer on his leg. Results of an evaluation of the histologic characteristics and the leukocytic immunophenotype of a skin biopsy specimen from the ulcer demonstrated large granular lymphocytes within the blood vessels. Cutaneous ulceration may be a manifestation of large granular lymphocytic leukemia, and this disease should be considered when diagnosing patients with otherwise unexplained pyoderma gangrenosum-like ulcers of the skin.
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PMID:Pyoderma gangrenosum-like ulcer in a patient with large granular lymphocytic leukemia. 146 48

A 19-year-old man with severe acne developed extensive scalp folliculitis and later superficial pyoderma gangrenosum following treatment with isotretinoin. A cyclical neutropenia was noted and bone marrow findings suggested myelodysplasia. We believe that isotretinoin was implicated in the development of overt symptoms in this patient whose haematological condition was previously asymptomatic.
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PMID:Development of folliculitis and pyoderma gangrenosum in association with abdominal pain in a patient following treatment with isotretinoin. 214 Dec 75

Atypical bullous pyoderma gangrenosum was diagnosed during the course of a myeloid malignancy in three patients. One patient had chronic myeloid leukaemia, one acute myeloid leukaemia, and the third, refractory anaemia with excess of blasts. This atypical form of pyoderma gangrenosum has been specifically associated with myeloid malignancies. The atypical appearance of the skin lesions and the clinical context in which they arose caused the true diagnosis to be delayed in all cases. Treatment with steroids was associated with rapid healing of the skin lesion. The histopathological changes in the skin biopsy specimens from these cases were non-specific, and although the histology was considered to be atypical of pyoderma gangrenosum in one case, the unusual features could be attributed to the patient's neutropenia. (Skin biopsy was performed to exclude other specific pathology). Atypical bullous pyoderma gangrenosum is an uncommon association of the myeloid malignancies. It may remain unrecognised and should be considered more often.
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PMID:Atypical bullous pyoderma gangrenosum associated with myeloid malignancies. 347 88

Granulocyte colony-stimulating factor (G-CSF) is a hematopoietic growth factor (HGF) with many applications in cancer therapy. The most important applications are reduction in the incidence of febrile neutropenia, acceleration of neutrophil recovery after chemotherapy or bone marrow transplantation, and mobilization of progenitor cells. Many cutaneous adverse reactions associated with HGF have been reported in recent years, including injection site reactions, pyoderma gangrenosum, Sweet's syndrome, cutaneous leucocytoclastic vasculitis, and widespread folliculitis. The presence of large histiocytes on the dermis between collagen bundles has been proposed as a characteristic histopathologic finding in cutaneous eruptions secondary to granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor. We report on a patient with a high-risk ductal infiltrating carcinoma of the breast who received high-dose chemotherapy (HDC) with peripheral blood progenitor cell (PBPC) rescue. The patient received G-CSF after PBPC for a faster granulocyte recovery. She developed a cutaneous eruption located on back, buttocks, axillae, groin and sites where electrocardiography electrodes had been placed. From the histopathological point of view, the eruption was characterized by the presence of numerous large, atypical histiocytes in the dermis with several mitotic figures, mimicking involvement of the dermis by a malignant process.
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PMID:Histopathology of cutaneous reaction to granulocyte colony-stimulating factor: another pseudomalignancy. 987 Jun 76

A 37-year-old African American female with a diagnosis of acute myelogenous leukemia (AML) being treated with chemotherapy presented with a lesion on her lower back within the confines of a newly inked tattoo. Five days after tattoo placement, she developed an oozing, indurated, necrotic plaque at the site. Four days later, she developed chills, fever, and neutropenia. A skin biopsy was performed and was consistent with pyoderma gangrenosum (PG) or neutrophilic dermatoses. PG is an inflammatory skin disease associated with both cutaneous trauma and systemic disease, including hematologic malignancy. PG after tattoo placement, in both healthy patients and those with hematologic malignancies, has, to our knowledge, not yet been described in the literature. While further studies are necessary to investigate the link between PG and tattooing, oncologists may wish to counsel patients with leukemia to refrain from obtaining new tattoos.
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PMID:Pyoderma gangrenosum following tattoo placement in a patient with acute myelogenous leukemia. 1827 27

We report the case of a 10-month-old girl who presented with a spontaneous ulcer on the left buttock which failed to heal despite antibiotic therapy. Histology showed changes consistent with pyoderma gangrenosum and the ulcer resolved rapidly with super-potent topical steroids under occlusion. Blood tests revealed a persistent neutropenia. Immunoglobulin G (IgG) antineutrophil antibodies were detected in the serum, directed against human neutrophil antigen (HNA)-1a. Bone marrow studies showed normocellular marrow with no evidence of dysplasia. T and B cell subsets and karyotype analysis were normal. Autoimmune neutropenia is an uncommon self-limiting condition in young children. Pyoderma gangrenosum is rare in infants, although the buttocks are a common site of involvement in this age group. Pyoderma gangrenosum in infancy can be associated with systemic disease as in adults, particularly myelodysplasia and leukemia, arthritis and inflammatory bowel disease. However, the association of pyoderma gangrenosum and autoimmune neutropenia of infancy has not previously been reported.
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PMID:Pyoderma gangrenosum in association with autoimmune neutropenia of infancy. 1906 67

We present a 24-year-old female referred with non-healing wound of a few days duration on anterior aspect of her right foreleg. Biopsy of the wound was reported to be pyoderma gangrenosum on pathologic report. Further work up of the patient for high grade fever and occasional leukopenias revealed the diagnosis of cyclic neutropenia. Treatment with granulocyte colony-stimulating factor (G-CSF) resulted in patient's neutrophil counts correction and dramatic improvement in healing of her lower extremity wound.
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PMID:A case report of cyclic neutropenia associated with pyoderma gangrenosum. 2213 Dec 49

We encountered a case of a man who was diagnosed with severe congenital neutropenia as a child and presented at the age of 45 years with pyoderma gangrenosum (PG) of the lower leg. PG associates with an underlying systemic disease, most commonly inflammatory bowel, rheumatic, or hematological disease or malignancy. However, in many cases, the underlying disease was not known. Surgery can trigger PG. The histopathological features of PG were nonspecific, and diagnosis requires excluding other conditions that have a similar appearance. Our analyses showed that the PG in our case was secondary to severe congenital neutropenia, which had promoted an infection of keratinous cysts. The patient bore a mutation in the ELANE gene encoding neutrophil elastase. Only 1 other case of neutropenia-associated PG has been reported previously: the association was only suspected. The present complex case was effectively treated by systemic treatment of the neutropenia with granulocyte colony-stimulating factor and regional surgical treatment. Histology of the excised tissue revealed keratinous cysts that were diffusely distributed with inflammatory granulation tissue. We believe that the rupture of the walls of the keratinous cysts may have caused the PG. At the time of writing (3 years since the initial presentation), the PG has not recurred. This case shows the importance of performing detailed examinations, including blood tests, to determine the disease underlying PG. This was because if the underlying disease was identified, its treatment was likely to promote healing of the wound after local surgery and prevent recurrence.
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PMID:Pyoderma Gangrenosum Secondary to Severe Congenital Neutropenia. 2970 46

Ecthyma gangrenosum (EG) is a severe potentially lethal cutaneous infection that progresses sequentially from maculopapular rash to haemorrhagic bulla and then to necrotic ulceration with surrounding erythema. It usually occurs in immunocompromised patients (aplasia secondary to chemotherapy, HIV infection, neutropenia or functional deficit of neutrophils, agammaglobulinemia). It rarely affects healthy people. Differential diagnosis includes leishmaniasis, pyoderma gangrenosum, eschars and papulonecrotic tuberculides. Blood cultures and/or local sample allow the isolation of P. aeruginosa, which is the causative germ. Treatment is based on suitable parenteral antibiotic therapy according to results of susceptibility tests (third-generation cephalosporins, fluoroquinolones). We here report the case of a 2-year old child with no particular past medical history presenting with multiple necrotic ulcers on the back with erythematous border evolving in a febrile context. Blood culture was negative. Laboratory tests showed sedimentation rate 30 mm, CRP 80mg/l. Bacteriological sampling of pus revealed pseudomonas aeruginosa. The diagnosis of ecthyma gangrenosum was made. The patient underwent parenteral third-generation cephalosporins. Complete patient recovery with healing occurred after 4 weeks.
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PMID:[Ecthyma gangrenosum]. 3034 79