Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pyoderma gangrenosum is a neutrophilic dermatosis of unknown aetiology. Visceral involvement by pyoderma gangrenosum is rare, the lung being the most frequent site of extracutaneous disease. We describe a 73-year-old man with pyoderma gangrenosum and chronic myelomonocytic leukaemia in whom aseptic hepatosplenic abscesses and bony lesions were associated.
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PMID:Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia. 1058 64

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a condition characterized by the sudden onset of fever, leukocytosis, and tender,erythematous, well-demarcated papules and plaques which show dense neutrophilic infiltrates on histologic examination. Although it ma occur in the absence of other known disease, Sweet's syndrome is often associated with hematologic disease (including leukemia), and immunologic disease (rheumatoid arthritis, inflammatory bowel disease). Treatment with systemic corticosteroids is usually successful. skin, Sweet syndrome, neutrophilic dermatosis, corticosteroids,
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PMID:Sweet's syndrome (acute febrile neutrophilic dermatosis). 1067 51

Psoriasis is a heterogenous skin disease, characterized by epidermal hyperproliferation, abnormal keratinization and inflammation. The heterogeneity of the disease results probably from the interaction of multiple gene abnormalities with environmental factors. The new approaches to drug design have become refocused to the emerging understanding of the role of signaling pathways in health and disease. Protein tyrosine kinases (PTKs) regulate cell proliferation, differentiation and immune processes. Uncontrolled signaling from receptor and intracellular tyrosine kinases can lead to numerous proliferative diseases: cancer, leukemia, restenosis and psoriasis. Identification of PTKs that play a key role in a defined disease can lead to a selective drug. The balance of signals which regulate the homeostasis of normal epidermis is altered in psoriasis. Several lines of evidence suggest a role for the EGF receptor system in this process. Therefore, blockers of the EGFR kinase were suggested as potent antipsoriasis agents. PTK inhibitors from the tyrphostin family were found to block EGF - dependent cell proliferation. AG 1571 (SU 5271) potently inhibits ligand-induced autophosphorylation of EGF-R, downstream signal transduction events, DNA replication and cell cycle progression at micromolar concentrations, as well as proliferation of keratinocytes isolated from psoriatic lesions in excellent correlation with its EGFR kinase inhibitory activity in these cells. AG 1571 (SU 5271) has been in clinical trials by SUGEN Inc. since early 1997. Overexpression of the EGFR is the hallmark of most epithelial cancers. Therefore one can view blockers of the EGFR kinase as becoming universal inhibitors. Tyrphostins are the first signal transduction agents to be used in the clinic. This article summarizes recent progress in the development of PTK inhibitors in the treatment of psoriasis.
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PMID:Inhibitors of tyrosine kinases in the treatment of psoriasis. 1082 17

The Human T-Cell lymphotropic virus type I (HTLV-I) is endemic in the Caribbean basin, Japan, Central Africa and South Pacific. It as been associated to Lymphoma-Leukemia of Adult T-Cells (ATLL) and a progressive spastic paraparesis (TSP/HAM). The nationwide seroprevalence of Panama is of 1-2%. We report a case of a 73 year old male, albino, single, patient, Panamanian descendent from Jamaican immigrants who presented a skin disorder which started 1 year ago, characterized by the appearance of infiltrative, intensely pruritic papules, nodules and a non exfoliative erythroderna involving face, neck, trunk and extremities. He also had painless enlarged cervical lymph nodes, non tender hepatomegaly. Laboratory studies revealed a keukocyte count of 128,000/ml wit 67% atypical lymphocytes, serum calcium was 12.5 mg/dl, DL in 583 UNI, "flower cells" and atypical lymphocytes with hyperlobulated nuclear contour was observed in the peripheral blood smear, seropositivity to TLV-I detected by enzyme-linked immunosorbent assay (Elisa) and confirmed by Western blot assay. The skin biopsy shows a bandlike dermal infiltrates of atypical lymphoid cells with epidermotropism and Pautrier's microabscesses. Once the treatment was initiated with prednisone, cyclophosphamide, and systemic antibiotics for a bronchopneumonic process most of the cutaneous lesions cleared up although the clinical condition of our patient became progressively worse and died after a acute renal failure and a lower gastrointestinal bleeding. In 1986, the tree first cases of ATLL were identified in Panama, there has not been apparently new cases reported until now.
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PMID:[T-cell lymphoma/leukemia secondary to HTLV-1 in adults. Report of a case]. 1099 95

Studies comparing functional differences in human T-cell leukemia virus type 1 (HTLV-1) clones that mediate distinct outcomes in experimentally infected rabbits, resulted in a dermatopathic smoldering adult T-cell leukemia/lymphoma following chronic infection with HTLV-1 strain RH/K34. During the 3.5 years' follow-up, HTLV-1 skin disease progressed to cutaneous T-cell lymphoma. When infection was passed to several naive rabbits, progressive paraparesis due to myelopathic neurodegeneration, analogous to HTLV-associated myelopathy, resulted in one of 4 transfusion recipients. Similar proviral loads were detected in the two diseases, regardless of stage of progression or tissue compartment of infection. Complete proviral sequences obtained from the donor and affected recipient aligned identically with each other and with the inoculated virus clone. Existence of disparate pathogenic outcomes following infectious transmission further extends the analogy of using rabbits to model human infection and disease. Although the experimental outcomes shown are limited by numbers of animals affected, they mimic the infrequency of HTLV-1 disease and authenticate epidemiological evidence of virus sequence stability regardless of disease phenotype. The findings suggest that further investigation of a possible role for HTLV-1 in some forms of cutaneous T-cell lymphoma is warranted.
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PMID:Passage of human T-cell leukemia virus type-1 during progression to cutaneous T-cell lymphoma results in myelopathic disease in an HTLV-1 infection model. 1100 4

Neutrophilic eccrine hidradenitis is a variety of neutrophilic dermatosis described in patients with different neoplasms, most often leukaemia, and different chemotherapy regimens. It is characterised by neutrophilic infiltration of the eccrine coils of sweat glands. Recently it has been described in healthy juveniles, involving primarily the soles of the feet. We describe five new cases of juvenile neutrophilic eccrine hidradenitis all showing a good prognosis or a self-limiting course.
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PMID:Juvenile neutrophilic eccrine hidradenitis: a vasculitis-like plantar dermatosis. 1114 61

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative disorder. Less than 50 cases have been reported. We report the first case of CNL with an associated leukemia cutis. CNL was diagnosed in a 74-year-old white woman in 1998, based on neutrophilic infiltration of the bone marrow and absence of the Philadelphia chromosome. The patient presented to the dermatology service in August 1998 with a 2-week history of a pruritic eruption on the arms, hands, and legs. Physical examination revealed red to violaceous plaques on both thighs and knees, in addition to purpuric patches and plaques on the dorsal hands, arms, and legs. Leukemia cutis was demonstrated on biopsy specimens of several lesional sites. The eruption progressed, despite treatment with topical and systemic corticosteroids. Treatment with systemic chemotherapy did affect partial resolution of the eruption, with parallel decreases in bone pain and white blood cell count, but the disease progressed and the patient ultimately died 5 months after her initial skin findings. Only one other case of CNL with dermatologic manifestations has been reported, CNL associated with a reactional neutrophilic dermatosis. Comparison to and differentiation from this case is discussed. The importance of distinguishing the specific infiltrates of leukemia from the nonspecific infiltrates of reactional dermatoses, such as Sweet's syndrome, is illustrated.
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PMID:Leukemia cutis in a patient with chronic neutrophilic leukemia. 1117 17

Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia. Clinical manifestations are polymorphous, and include erythema, bullae, erythema multiforme-like lesions and severe mucous membrane involvement. PNP manifesting as lichenoid dermatitis has recently been observed. We describe two Italian men with fatal PNP featuring typical PNP autoantigens associated with chronic B-cell lymphocytic leukaemia. The first patient presented with an extensive blistering eruption, several erythema multiforme-like lesions and severe mucosal involvement. The second patient presented with a lichenoid dermatitis, then developed bullae, and died with an erythrodermic and exfoliative dermatosis resembling pemphigus foliaceus. Our patients represent two Italian cases of well-documented PNP. In patient 2, the sequence of clinical presentations was unique, and strongly supports the hypothesis of epitope spreading through chronic lichenoid inflammation of the dermo-epidermal junction exposing new self antigens, leading to the humoral response characteristic of PNP.
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PMID:Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia. 1145 21

Sweet's syndrome (SS), or acute febrile neutrophilic dermatosis, is a condition characterized by the sudden onset of fever, leukocytosis, and painful, erythematous, well-demarcated papules and plaques which show dense, neutrophilic infiltrates on histologic examination. Myalgias and arthralgias are common. Although it may occur in the absence of other known disease, SS is often associated with hematologic disease (including leukemia), and immunologic disease (rheumatoid arthritis, inflammatory bowel disease). A case of SS is reported. Furthermore the patient presented multiple intestinal lipomas and diverticular colon disease. The authors, on the ground of recent studies which correlate SS with several digestive system disorders, hypothesize an association between acute febrile neutrophilic dermatosis and patient's intestinal diseases. Treatment with systemic corticosteroids is usually successful.
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PMID:[Sweet syndrome in a female patient with intestinal multiple lipomas and diverticular disease of the colon]. 1169 4

A 20-year-old woman presented with a 4-month history of follicular papules distributed over the trunk and extremities. One month later, routine blood tests were abnormal, showing acute myeloblastic leukaemia (M1 in the French-American-British classification). Skin biopsy demonstrated a dermal infiltrate of a large number of neutrophils with occasional eosinophils and histiocytes in the vicinity of the hair follicle remnants. Intermingled in the infiltrate were atypical cells that were morphologically and immunohistochemically identical to leukaemic myeloblasts. Cultures of the papules and special stains of the biopsy specimen were negative for bacteria and fungi. The follicular eruption improved promptly in response to chemotherapy for the leukaemia. We suggest that this case may represent a rare, follicular variant of neutrophilic dermatosis associated with myelogenous leukaemia.
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PMID:Sterile suppurative folliculitis associated with acute myeloblastic leukaemia. 1200 Mar 94


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