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)

A 23-year-old man is reported who had a connective tissue disorder with features of rheumatoid arthritis and systemic lupus erythematosus. Because of the development of severe myocarditis and acute cerebral dysfunction treatment with azathioprine and prednisone was instituted, with dramatic clinical improvement. Ten months later, when a total of some 52 g of azathioprine had been administered, acute myelomonocytic leukemia developed. Although acute myelogenous leukemia has been noted in several individuals receiving immunosuppressive drugs for treatment of lymphoreticular neoplasms, this complication has heretofore been rare in patients given azathioprine for non-neoplastic disease. The present case is documented because of the increasing use of immunosuppressive drugs in treatment of various rheumatic disorders and the need to establish the relationship, if any, between the use of such agents and malignancy.
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PMID:Acute leukemia after azathioprine treatment of connective tissue disease. 26 41

A 68-year-old man with haemotological features consistent with haemopoietic dysplasia (pre-leukaemia) and an abnormal cell clone in the marrow, 46, XY, -18, +t(13;18) (q11;123), developed acute connective tissue disease characterised by vasculitis, dermal changes, marked muscular weakness and serological features suggesting an auto-immune disturbance. Although four other cases of haemopoietic dysplasia ("pre-leukaemia") with unusual connective tissue disease had been reported since the recognition of haemopoietic dysplasia as a distinct entity, a definite association between these two disease states still awaits confirmation by further reports and investigations.
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PMID:The association of haemopoietic dysplasia (pre-leukaemia) and abnormal cell clone with connective tissue disease. 28 61

A series of 122 consecutive patients with bone marrow fibrosis initially referred or categorized as idiopathic myelofibrosis is described. After a clinical and pathological review 14 patients were classified as postpolycythaemic myelofibrosis and 7 patients as a transitional myeloproliferative disorder. In 13 patients a diagnosis of hairy cell leukaemia was made, 3 patients had malignant lymphoma, 2 had malignant histiocytosis, and 1 patient had systemic lupus erythematosus with myelofibrosis. Two patients were excluded for further analysis owing to insufficient data. In the remaining 80 patients a diagnosis of idiopathic myelofibrosis was made. The clinical and laboratory findings in this series of patients are presented and compared to those in previous series. Infectious, cardiovascular, thromboembolic, and haemorrhagic complications were frequent, being recorded in 63%, 50%, 40%, and 33% of the patients, respectively. Various autoimmune phenomena were found in a proportion of the patients, but none had clinical evidence of connective tissue disease. Fifteen patients (19%) had a syndrome of acute myelofibrosis. The diagnostic criteria for this disease entity and its place within the spectrum of myeloproliferative disorders are discussed. In the present series acute myelofibrosis was found to encompass various transitional stages toward the evolution of acute leukaemia. It is proposed that acute or malignant myelofibrosis is considered as an acute variant of idiopathic myelofibrosis. Within this syndrome the acute variant seems to be far more common than previously recognized, which may also explain the marked clinical heterogeneity of the myelofibrosis/osteomyelosclerosis syndrome in this and most previous series.
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PMID:Idiopathic myelofibrosis: a clinical study of 80 patients. 219 69

cDNA encoding a 70 kd protein (70K) associated with U1 small nuclear ribonucleoprotein (snRNP) was cloned from a human brain-stem library using autoantibodies from patients with connective tissue disease. The cDNA-derived amino acid sequence contains 23 residues homologous to a region of murine leukemia virus group-specific antigen p30gag. The homology residues in an antigenic portion of the 70K protein and is defined by a core consensus sequence, ETPEEREERRR, that occurs as a tandem repeat in p30gag of most mammalian type C retroviruses. Anti-p30gag antibodies recognized a recombinant 70K-LacZ fusion protein as well as U1 snRNPs. Using synthetic peptides as competitors, we demonstrated that the region of homology encompasses the site of immunological cross-reactivity. Thus autoantibodies against U1 snRNPs were elicited by immunization with p30gag. On the basis of these findings, we suggest a role for retroviruses in the initiation of autoimmunity.
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PMID:A human autoimmune protein associated with U1 RNA contains a region of homology that is cross-reactive with retroviral p30gag antigen. 295 71

Periarteritis Nodosa (P.A.N.) is a systemic connective tissue disease with a variety of manifestations that includes ocular involvement in 20% of cases. The diagnosis of this condition is difficult due to the absence of any specific clinical signs or laboratory findings. However, histologic studies have demonstrated a segmental vasculitis that is often necrotic. Ocular findings frequently include choroidal involvement that is characteristic. Nevertheless, angiographic studies of this disease are extremely rare. The findings in three patients suspected of having P.A.N. are presented. Fluorescein angiography established the diagnosis of P.A.N. in two cases and ruled-out its presence in the third case. In the first case angiography demonstrated a retinal vasculitis with multiple arteriolar and capillary occlusions. There was also ischemic involvement of the choriocapillaris and a mild anterior optic nerve vasculitis. All findings resolved, leaving numerous Elschnig spots. In the second case the angiogram showed acute multifocal ischemia of the choriocapillaris. The ocular examination and fluorescein angiogram in the third case were entirely normal, thereby ruling-out P.A.N. on the basis of insufficient criteria. Acute multifocal choroidal ischemia is present in a variety of rare conditions: Toxemia of pregnancy, Disseminated Intravascular coagulopathy, Moskowitz Disease (T.T.P.), Leukemia and Malignant Hypertension. However, the presence of multifocal choroidal ischemia in the presence of a systemic connective tissue disorder strongly favors the diagnosis of P.A.N. The relative contributions of co-existent Malignant Hypertension and P.A.N. in producing choroidal ischemia are discussed. The spectrum of clinical manifestations and laboratory findings in P.A.N. as well as hypotheses concerning pathogenesis (immune-complex deposition) are described. Among all systemic vasculitis , only P.A.N., and rarely Scleroderma, feature choroidal involvement. This is possibly due to the fact that the degree of vasculitis in P.A.N. is sufficiently severe to cause clinically significant choroidal involvement.
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PMID:[Fluorescein angiography in the diagnosis of periarteritis nodosa]. 614 75

A patient is reported, in whom clinical and biochemical evidence of connective tissue disease preceded the diagnosis of hairy-cell leukaemia by at least 2 years. The pathogenetic mechanism(s) responsible for the coexistence of hairy-cell leukaemia with vasculitis and rheumatic disease is discussed. It is proposed that the 2 diseases may share a common predisposing factor, or that the hairy cells may elicit disturbances in immune homeostasis, implying a dysfunction of T suppressor cells. Hairy-cell leukaemia should be considered in the differential diagnosis in cases of unexplained febrile illness associated with pancytopenia and signs of connective tissue disease.
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PMID:Hairy-cell leukaemia simulating connective tissue disease. 672 97

Retroviral diagnostics have become standard in human laboratory medicine. While current emphasis is placed on the human exogenous viruses (human immunodeficiency virus and human T-cell leukemia virus), evidence implicating human endogenous retroviruses (HERVs) in various human disease entities continues to mount. Literature on the occurrence of HERVs in human tissues and cells was analyzed. Substantial evidence documents that retrovirus particles were clearly demonstrable in various tissues and cells in both health and disease and were abundant in the placenta and that their occurrence could be implicated in some of the reproductive diseases. The characteristics of HERVs are summarized, mechanisms of replication and regulation are outlined, and the consistent hormonal responsiveness of HERVs is noted. Clear evidence implicating HERV gene products as participants in glomerulonephritis in some cases of systemic lupus erythematosus is adduced. Data implicating HERVs as etiologic factors in reproductive diseases, in some of the autoimmune diseases, in some forms of rheumatoid arthritis and connective tissue disease, in psoriasis, and in some of the inflammatory neurologic diseases are reviewed. The current major needs are to improve methods for HERV detection, to identify the most appropriate HERV prototypes, and to develop diagnostic reagents so that the putative biologic and pathologic roles of HERVs can be better evaluated.
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PMID:Human endogenous retroviruses: nature, occurrence, and clinical implications in human disease. 889 53

Cytopenias are typical of patients with connective tissue disease (CTD) and are usually related to autoimmune phenomena. In some cases, cytopenia may be the result of treatment with cytotoxic agents. Although multi-drug therapy is known to produce myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML) in patients with CTD, treatment with single-agent therapy, particularly methotrexate, has rarely been associated with secondary MDS or AML. Blood and marrow samples were studied from 3 men and 5 women with rheumatoid arthritis (5 cases), Behcet's disease (2 cases), and systemic lupus erythematosus (1 case) developing MDS or AML after methotrexate (5 cases), chlorambucil (2 cases), and cytoxan (1 case). The durations of CTD ranged from less than 6 months to more than 10 years. Five patients (63%) presented with MDS including refractory anemia (RA), refractory thrombocytopenia (RT), refractory anemia with excess blasts (RAEB), chronic myelomonocytic leukemia (CMML), and RAEB in transformation. Patients with RT, CMML, and RAEB in transformation developed AML. Of six patients presenting with or developing AML, four had AML with differentiation (FAB M2), one acute myelomonocytic leukemia (FAB M4), and one M4Eo. Inv 16 was seen in the M4Eo and t(8;21) in one case of M2. Four of six patients are alive up to 6 years after diagnosis of AML. One of three patients with MDS is alive 6 months after diagnosis of MDS. Cytopenias in patients with CTD may be due to therapy-related MDS or AML occurring in a setting of single-agent chemotherapy, including methotrexate.
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PMID:Myelodysplastic syndromes and acute myeloid leukemia in connective tissue disease after single-agent chemotherapy. 892 81

In a prospective study conducted in the Institute of Postgraduate Medicine & Research (IPGMR), Dhaka, 212 patients with prolonged pyrexia were thoroughly evaluated clinically and with the help of laboratory investigations with a view to reaching the diagnosis. Their clinical and laboratory data were recorded. Clinical features pertaining to a particular organ gave appropriate clue in 52% cases. Imaging techniques were instrumental in 24%, microbiological or serological investigations in 35%, invasive procedures were diagnostic in 42%, laparotomy had to be resorted to in five cases. Infectious diseases were the commonest causes of prolonged pyrexia accounting for about 63.21% of cases followed by neoplasms (12.74%) and connective tissue disorders (10.85%). Tuberculosis was the most common infection (24.53% of all cases) followed by enteric fever (12.74%) and visceral leishmaniasis (9.43%). Pleura was the commonest seat for tuberculosis followed by lymph nodes and abdomen. Leukemias were the commonest neoplasm and SLE the commonest connective tissue disorder presenting with prolonged fever. Several fundamental observations were made in the study. Infections are the commonest cause of prolonged fever in our community, neoplasms and connective tissue disorders are also not rare. Secondly, patients with temperature between 100 to 101 degrees F should not be denied evaluation with the apprehension of unnecessarily investigating for habitual hyperthermia, as the condition was distinctly rare in the series. Thirdly, analysis of materials from organs or systems suspected to be abnormal clinically or by simple imaging techniques had high diagnostic yield. Finally, usual causes of prolonged fever are illnesses ordinarily encountered in clinical practice, pyrexia becomes protracted either because the presentation is atypical or incomplete, or because we fail to make proper use of available clinical or paraclinical information.
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PMID:A study of prolonged pyrexia in Dhaka. 903 43

There is a growing body of information about the soluble forms of HLA in serum but there are only a few reports discussing sHLA in other body fluids. We quantitated sHLA-I and sHLA-II concentrations in sweat, saliva and tear samples from five normal individuals with known HLA-phenotypes. We also studied sweat samples from an additional 12 normal nonphenotyped subjects, as well as in CSF of 20 subjects with different illnesses, using solid phase enzyme linked immunoassay. Sweat, saliva and tears from normal subjects were found to contain very low or nondetectable amounts of sHLA-I. In contrast, sHLA-II molecules were found in each of these body fluids, although, with considerable variation between individuals. The presence of sHLA-II in saliva was further confirmed by Western-blotting. It was observed that sHLA-II having molecular mass of 43,900 and 18,100 daltons was comparable with that found in serum from normal individuals. In addition, no association of sHLA-II levels with allospecificities in either body fluid or in serum was apparent. The results of CSF sHLA concentrations in different diseases were as follows: (1) High CSF SHLA-I levels were measured during viral encephylitis (n = 3), while none of these patients contained sHLA-II in CSF; (2) The levels of sHLA-II, but not sHLA-I were elevated in CSF of patients during seizure (n = 6) and of patients with neonatal hepatitis (1 of 2) or with connective tissue disease accompanied with viral infection (n = 2); (3) No CSF sHLA-I or sHLA-II could be detected at polyneuropathy (n = 2), or in patients with syphilis (n = 3), or leukemia (n = 2) with evidence of neurologic involvement of central nervous system. Taken together, it may be concluded that the presence of sHLA in several body fluids is physiologically normal. It appears that sHLA-II is the predominant class of HLA molecules present in different body fluids. We propose that the system responsible for sHLA-II production in various body fluids must involve different mechanisms than those responsible for sHLA-I synthesis in serum.
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PMID:Soluble HLA in human body fluids. 1032 60


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