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)

Authors report a case in which relapsing polychondritis had been diagnosed two years before myelodysplastic syndrome developed and terminated in eosinophilic leukemia. The observation that relapsing polychondritis may precede myelodysplasia is not in concordance with some of the previous reports regarding relapsing polychondritis as a paraneoplastic phenomenon of myelodysplastic syndrome. The terminally developed eosinophilic leukemia is not supposed to be a blastic phase of the underlying myelodysplasia, much rather a second malignant process. This opinion may be confirmed by the early presence of blast cells in the myelodysplastic process without eosinophilia. It seems interesting to note that both our patient and his daughter suffered from diseases of autoimmune origin: acquired vitiligo and subacute cutan lupus erythematodes, respectively.
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PMID:[Relapsing polychondritis associated with myelodysplasia and acute eosinophilic leukemia]. 917 85

Vitiligo is an autoimmune disease caused by T-lymphocyte-mediated destruction of melanocytes. We describe two patients with generalized vitiligo caused iatrogenically after donor lymphocyte infusion (DLI) for leukaemia relapse over 3 years after bone marrow transplantation (BMT). Neither the sibling donor nor the recipient had vitiligo or other autoimmune diseases, and vitiligo did not occur after the first BMT. DLI was accompanied by skin graft-versus-host disease in both cases, which was controlled with immunosuppression. However, over several months, progressive generalized and persistent skin depigmentation occurred in both patients. Peripheral blood molecular studies showed the complete disappearance of host haematolymphopoiesis. The specific destruction of melanocytes in both patients was therefore probably mediated by new alloreactive lymphocytes infused from the donors.
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PMID:Generalized vitiligo after lymphocyte infusion for relapsed leukaemia. 1189 25

The aim of the present study was to determine whether angelicin is able to increase the expression of gamma-globin genes in human erythroid cells. Angelicin is structurally related to psoralens, a well-known chemical class of photosensitizers used for their antiproliferative activity in treatment of different skin diseases (i.e., psoriasis and vitiligo). To verify the activity of angelicin, we employed two experimental cell systems, the human leukemic K562 cell line and the two-phase liquid culture of human erythroid progenitors isolated from normal donors. The results of our investigation suggest that angelicin, compared with cytosine arabinoside, mithramycin and cisplatin, is a powerful inducer of erythroid differentiation and gamma-globin mRNA accumulation of human leukemia K562 cells. In addition, when normal human erythroid precursors were cultured in the presence of angelicin, increases of gamma-globin mRNA accumulation and fetal hemoglobin (HbF) production, even higher than those obtained using hydroxyurea, were detected. These results could have practical relevance, as pharmacologically-mediated regulation of the expression of human gamma-globin genes, leading to HbF induction, is considered a potential therapeutic approach in hematological disorders, including beta-thalassemia and sickle cell anemia.
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PMID:Accumulation of gamma-globin mRNA in human erythroid cells treated with angelicin. 1293 Mar 20

T cells responsive to minor histocompatibility (H) antigens are extremely effective in curing leukemia but it remains unknown whether they can eradicate solid tumors. We report that injection of CD8(+) T cells primed against the immunodominant H7(a) minor H antigen can cure established melanomas in mice. Tumor rejection was initiated by preferential extravasation at the tumor site of interferon (IFN)-gamma-producing H7(a)-specific T cells. Intratumoral release of IFN-gamma had two crucial effects: inhibition of tumor angiogenesis and upregulation of major histocompatibility complex (MHC) class I expression on tumor cells. Despite ubiquitous expression of H7(a), dissemination of a few H7(a)-specific T cells in extralymphoid organs caused neither graft-versus-host disease (GVHD) nor vitiligo because host nonhematopoietic cells were protected by their low expression of MHC class I. Our preclinical model yields unique insights into how minor H antigen-based immunotherapy could be used to treat human solid tumors.
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PMID:T cells targeted against a single minor histocompatibility antigen can cure solid tumors. 1622 89

Chronic myelomonocytic leukemia (CMML) is a hematologic stem cell disorder with myelodysplastic and myeloproliferative characteristics. Extramedullary leukemic infiltration of the skin, although uncommon in CMML, has prognostic relevance. We report a unique case of a patient with CMML who clinically presented with vitiligo that was histologically diagnosed as leukemia cutis. This case underscores the necessity of early recognition of skin changes in patients with hematologic disorders and the differentiation of nonspecific skin lesions from leukemia cutis.
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PMID:Leukemia cutis masquerading as vitiligo. 1844 70

Human T-cell lymphotropic virus (HTLV) types 1 and 2 belong to the Oncorna group of retroviridae, a large family of viruses, grouped initially by pathogenic features, but later revised on the basis of genome structure and nucleotide sequence. HTLV-I was the first discovered human retrovirus to be associated with a malignancy in 1980. The malignancy, first described by Uchiyama and co-workers in southwestern Japan, was named Adult T-cell Leukemia/Lymphoma (ATL) and characterized with cutaneous and respiratory involvement, hepatosplenomegaly, lymphadenopathy and various metabolic abnormalities such as hypercalcemia. The HTLV-I has been known to be endemic to certain parts of Iran like the province of Khorasan in the northeast since 1990, with a 2.3% prevalence rate of infection. The main manifestations of HTLV-I infection are neurologic and hematologic (such as ATL) disorders, but it has also other manifestations such as uveitis, arthritis, dermatitis, vitiligo and lymphocytic alveolitis. Its main neurologic manifestation is a chronic progressive myelopathy that is referred to HTLV-I Associated Myelopathy (HAM) in Japan and Tropical Spastic Paraparesis (TSP) in Caribbean. But other disorders such as peripheral neuropathy, polyradiculoneuropathy, myopathy, peripheral facial paresis, and so on have been reported too. In this review we wish to give some brief information on the different aspects (including epidemiology, pathogenesis and pathology, clinical findings, and treatment) of HTLV-I infection according to our twenty-year researches. The department of neurology of Mashhad University of Medical Sciences has been a pioneer in researches on HTLV-I in the last twenty years.
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PMID:"HTLV-I Infection" Twenty-Year Research in Neurology Department of Mashhad University of Medical Sciences. 2447 Aug 62

Genetic linkage analyses, genome-wide association studies of single nucleotide polymorphisms, copy number variation surveys, and mutation screenings found the human chromosomal 12q24 locus, with the genes SH2B3 and ATXN2 in its core, to be associated with an exceptionally wide spectrum of disease susceptibilities. Hematopoietic traits of red and white blood cells (like erythrocytosis and myeloproliferative disease), autoimmune disorders (like type 1 diabetes, coeliac disease, juvenile idiopathic arthritis, rheumatoid arthritis, thrombotic antiphospholipid syndrome, lupus erythematosus, multiple sclerosis, hypothyroidism and vitiligo), also vascular pathology (like kidney glomerular filtration rate deficits, serum urate levels, plasma beta-2-microglobulin levels, retinal microcirculation problems, diastolic and systolic blood pressure and hypertension, cardiovascular infarction), furthermore obesity, neurodegenerative conditions (like the polyglutamine-expansion disorder spinocerebellar ataxia type 2, Parkinson's disease, the motor-neuron disease amyotrophic lateral sclerosis, and progressive supranuclear palsy), and finally longevity were reported. Now it is important to clarify, in which ways the loss or gain of function of the locally encoded proteins SH2B3/LNK and ataxin-2, respectively, contribute to these polygenic health problems. SH2B3/LNK is known to repress the JAK2/ABL1 dependent proliferation of white blood cells. Its null mutations in human and mouse are triggers of autoimmune traits and leukemia (acute lymphoblastic leukemia or chronic myeloid leukemia-like), while missense mutations were found in erythrocytosis-1 patients. Ataxin-2 is known to act on RNA-processing and trophic receptor internalization. While its polyglutamine-expansion mediated gain-of-function causes neuronal atrophy in human and mouse, its deletion leads to obesity and insulin resistance in mice. Thus, it is conceivable that the polygenic pathogenesis of type 1 diabetes is enhanced by an SH2B3-dysregulation-mediated predisposition to autoimmune diseases that conspires with an ATXN2-deficiency-mediated predisposition to lipid and glucose metabolism pathology.
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PMID:12q24 locus association with type 1 diabetes: SH2B3 or ATXN2? 2493 53

With an incidence of 1 in 700 births, Down syndrome (DS) is not an uncommon condition. It is associated with various disorders of different organ systems. Serious disorders include cardiac defects and leukemia. With an onset during the newborn period, the latter does not always progress to classic myeloid leukemia (transient myeloproliferative disorder). Skin manifestations in newborns include pustules/vesiculopustules. In individuals with DS, such lesions should not only prompt suspicion for typical neonatal rashes and infections but also for transient myeloproliferative disorder. However, most dermatoses are benign. They essentially comprise disorders of keratinization that present as xerosis, keratosis pilaris, lichenification, and ichthyosis vulgaris. Also typical but not specific is the four-finger palmar crease (simian crease). Patients frequently develop folliculitides, which - due to elastolysis - subsequently progress to anetoderma. The known immune disturbance in DS patients explains the occurrence of autoimmune diseases such as alopecia areata and vitiligo. Typical skin conditions associated with DS include elastosis perforans serpiginosa, syringomas, milia-like calcinosis cutis, and multiple eruptive dermatofibromas.
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PMID:Dermatological aspects of the S2k guidelines on Down syndrome in childhood and adolescence. 3030 Apr 91

Lichen sclerosus (LS), also known as lichen sclerosus et atrophicus, is a chronic inflammatory mucocutaneous disease affecting the genital and/or extragenital areas. Although LS usually occurs alone, its coexistence in morphea patients has been reported in 5.7% and 38.0% (genital LS) of cases, in two series (1). A 74-year-old woman presented with a 6-month history of multiple asymptomatic, shiny , indurated, brownish large flat plaques located on the abdomen (Figure 1, a-b) and back, intermingled with slightly atrophic, white-colored, guttate, and patchy areas (Figure 1, d-e). Both punch biopsies of the sclerotic plaques on the back and abdomen showed findings consistent with morphea (Figure 1, c, f). Furthermore, the punch biopsy of a well-demarcated white plaque on the back revealed findings compatible with LS (Figure 1, f). Remarkably, there were also multiple white-colored lesions on the sites of pregnancy-induced striae distensae (SD) (Figure 2, a-b) on the lower abdomen and an old appendectomy scar (Figure 2, c). There was no anogenital involvement. A diagnosis of morphea-LS overlap was established and white lesions located on the surgical scar and SD were clinically evaluated as LS. Methotrexate (15 mg/week) achieved a partial regression of morphea plaques in three months. However, white LS lesions remained unchanged. Our patient presented with coexistence of LS and morphea on different sites of the trunk and on the same lesion. Additionally, one of the isolated LS lesions was located on a surgical scar. Occurrence of LS on skin grafts, irradiated areas, injection sites, or burn/surgical scars has been attributed to the Koebner phenomenon, also called isomorphic response, defined as "the formation of the skin lesions in the same morphology of the existing disease on the areas of various cutaneous injuries" (2). LS is classified under the Koebner category-III (occasional lesions) (2). However, in a case of morphea with features of LS that developed in 1 month following a herpes zoster infection has been suggested to represent "Wolf's isotopic response" (3), which was originally defined as "the occurrence of a new skin disease at the site of another, unrelated and already healed skin disorder" with a time interval between the first and second diseases ranging from months to several years (4). Remarkably, typical morphea plaques in our patient did not involve the surgical scar, in contrast to a cohort in which 16% of 329 patients developed initial morphea lesions at sites of prior (surgery) or ongoing/repetitive (chronic friction) skin trauma (5). SD appear on skin as atrophic linear bands mostly due to rapid weight changes, pregnancy, Cushing syndrome, or prolonged use of corticosteroids (6). The mechanism underlying the occurrence of several diseases on striae is still elusive. Blunt trauma occurring during the development of striae has been suggested to cause the Koebner phenomenon in patients with vitiligo, psoriasis, and lichen planus (7), but it has been suggested that the occurrence of leukemia cutis on SD in a patient reflects Wolf's isotopic response (8). Although chronic graft-versus-host disease, urticarial vasculitis, keloid, lupus erythematosus, diffuse normolipemic plane xanthoma, and drug-induced cutaneous eruptions have been reported to occur on striae (6,9), such an association with LS as in our patient has not been previously documented in the literature. Concomitant occurrence of LS patches on different previous lesions such as a surgical scar and SD in our patient raises the possibility of a common underlying mechanism. As mentioned above, the terms "Koebner phenomenon" or "Wolf's isotopic response" have been used to designate the development of some diseases in injured areas. However, Happle and Kluger (10) claimed in a recent statement that "there is no clear-cut criterion to distinguish isotopic response from Koebner phenomenon and all reactions of this kind represent examples of Koebner phenomenon", which seems to be the best way to describe the site-specific occurrence of LS lesions in our patient.
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PMID:Lichen Sclerosus on the Sites of Striae Distensae and a Surgical Scar in a Patient with Coexistent Morphea. 3103 93