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

Two brothers developed acute leukemia, one at the age of 7 months and the other at the age of 14 months. Both suffered from a staturoponderal retardation and the same malformation syndrome. The karyotype carried out only on the second child revealed breaks and chromatid changes. A diagnosis of Fanconi's anaemia can be discarbed since no blood cytopenia preceded the leukemia. Finally, the diagnosis of Bloom's syndrome prevailed despite the absence of telangiectatic erythema and the atypical chromosomal anomalies.
 ...
PMID:[Bloom's syndrome. Discussion of the diagnosis concerning two cases of terminal leukemia in a sibship (author's transl)]. 59 25

Leukopenia or pancytopenia as a result of bone marrow dysfunction are manifestations of various diseases or complications of therapeutic regimens. The spectrum of diseases associated with leukopenia is wide and includes congenital as well as acquired neutropenias secondary to conditions such as myelodysplastic syndromes, AIDS, malignant tumors with or without chemotherapy-enhanced neutropenia, bone marrow transplantation or therapeutic or accidental radiation. The morbidity and mortality of infectious diseases is greatly enhanced during neutropenic phases. Over the last few years attempts have been made to shorten the duration and lessen the severity of neutropenia in patients with the above conditions by administration of Granulocyte Macrophage Colony Stimulating Factor (G-CSF). Both cytokines were successfully tested in phase I and II trials. Treatment with GM-CSF or G-CSF results in a dose-dependent increase of the neutrophil count. GM-CSF also increases the number of eosinophils and monocytes in peripheral blood. The effect of both cytokines on the neutrophil count is transient as long as the underlying disease persists. This prompted the institution of maintenance therapy, which has been successfully used with either cytokine. Long-term treatment is usually well tolerated and results in a reduction in the frequency of infections as well as in the duration of antibiotic treatments. Side effects of GM-CSF or G-CSF are usually mild and include fever, myalgia, bone pain, and erythema. A number of patients developed dyspnea, hypotension, sweating, flushing and erythema after the first dose of GM-CSF in each treatment cycle. This first-dose reaction occurs more frequently after intravenous than reactions were reported with G-CSF. Some patients with myelodysplastic syndrome progressed to acute myeloic leukemia during or after treatment with GM-CSF or G-CSF. Most of these patients presented with an increased fraction of blasts in the bone marrow, which preceded the treatment with the colony stimulating factors. Since GM-CSF and possibly G-CSF may increase the risk of developing acute leukemia in patients with myelodysplastic syndrome, it appears prudent to limit the use of these cytokines in patients with this disease. The subcutaneous route of administration appears to be preferable to intravenous administration, since the incidence and severity of side effects are reduced. While many questions concerning dosage, long-term therapy and combination therapy still remain unanswered, the information presented in this review concerning the clinical use of these cytokines warrants an optimistic outlook.
 ...
PMID:[GM-CSF and G-CSF: cytokines in clinical application]. 170 94

We describe a patient with acute myelomonocytic leukemia who demonstrated leukemic cell infiltration to scratched wounds and scars from trauma. A 65-year-old Japanese woman developed low grade fever, headache and exanthema. Hematology testing disclosed leukocytosis of 95,600/mm3 with 65% monocytes and 9% blast cells. Infiltrated erythema and nodules were disseminated over most of her body. Moreover, linear scratched wounds and traumatic scars were indurated. Skin biopsy showed dense atypical mononuclear cell infiltration with monocytic characteristics. We discuss the possible reasons for the infiltration of leukemia cells into the wounds and scars from trauma.
 ...
PMID:Leukemia cutis in acute myelomonocytic leukemia: infiltration to minor traumas and scars. 193 54

In a previous study on immune responsiveness among asymptomatic human T-cell leukemia virus type I (HTLV-I) carriers, we found that carriers had significantly reduced delayed-type hypersensitivity (DTH) response to purified protein derivative (PPD) skin testing. The association was strongest among persons at least 60 years of age. In order to evaluate this finding further, we evaluated the response to both PPD and phytohemagglutinin (PHA) in an elderly population. Fifty-six consecutive hospitalized patients with nonimmunosuppressive diseases were examined. None had a history of tuberculosis nor evidence of the known HTLV-I-associated diseases. The subjects' ages ranged from 62-93 years (median = 75 years); 43 were women and 13 were men. Twenty-two of the subjects were HTLV-I antibody positive. Among the carriers, there was an increased level of nonreactivity to PPD, the relative risk adjusted for age (RR) being 1.9 (95% confidence interval, 0.62-5.8), as well as to PHA of RR = 2.3 (0.60-9.0). When subjects were cross-classified for response to both skin tests, 15 of 17 carriers were nonreactive to either or both antigens compared to 15 of 25 noncarriers [RR = 5.1 (0.99-25.9) (p value, one-sided = 0.026)]. The decline in reactivity to both antigens increased with age, but was consistently lower among the carriers. Among subjects with positive reactions to PPD, the degree of reaction as measured by the size of erythema was reduced among the carriers; however, for PHA responders, the response in carriers appeared to be normal. Among the HTLV-I antibody negative subjects, the size of erythema for both antigens was strongly correlated (p = 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Suppression of delayed-type hypersensitivity to PPD and PHA in elderly HTLV-I carriers. 239 53

"Congenital telangiectatic erythema" (Bloom's syndrome) is very rare; it is linked to a group of hereditary diseases and a common feature is cellular hypersensitivity to a variety of physical or chemical agents. The mode of transmission is autosomal-recessive. Characteristic criteria for Bloom syndrome are: consanguinity of the parents; androtropia; low birth weight, short stature (proportional); and persistent telangiectatic erythema in sun-exposed areas, sometimes with blistering. The syndrome is also associated with a facultative lack of antibodies. Of great importance is the high leukaemia morbidity among individuals with this syndrome; chromosomal aberrations and breakages play a significant role. Histological changes comprise an increase in dilated vessels in the upper dermis and damage and loss of elastic fibers. We give a review of Bloom's syndrome and present a case report.
 ...
PMID:[Congenital telangiectatic erythema (Bloom syndrome)]. 304 5

A 4-month-old infant was admitted with a monoblastic infiltration of the skin associated with osteosclerosis. Both lesions spontaneously disappeared within a few months, but 2 years later, a monoblastic leukemia occurred that was associated with marked skin erythema and myelofibrosis. Skin and bone marrow specimens showed a monoblastic infiltration with numerous intermingled mast cells of normal appearance. Whether myelofibrosis was a feature of a systemic mastocytosis or of the leukemic process is discussed in this case.
 ...
PMID:Acute monoblastic leukemia with osteosclerosis and extensive myelofibrosis. 323 9

Soft tissue infections were seen in 25 patients with underlying malignancy and immunosuppressive disease. The primary disease included leukemia, lupus, aplastic anemia, lymphoma, carcinoma and myeloma. Infectious sites included the perianal area, gluteal, chest wall, extremity and the vulva. Eighty per cent of the infectious episodes occurred in patients who were granulocytopenic. Initial presentation was of local tenderness and redness. Fluctuation and discoloration were present in nine patients who were also hypotensive. Local drainage in five patients resulted in the death of two (20%). Overall, the mortality was 3/25 (12%). Wide debridement and drainage and appropriate antibiotic therapy resulted in the death of 1/20 (5%) patients. Hypotension, discoloration and fluctuation were found to be late signs in these patients. Soft tissue infections in the compromised host present subtly and progress to death if treatment is delayed. Temperature elevation and localized tenderness and erythema are indications for broad spectrum antibiotics and extensive intraoperative drainage and debridement.
 ...
PMID:Soft tissue infections in the compromised host. 338 98

Angioimmunoblastic lymphadenopathy often begins with constitutional symptoms, such as fever, malaise, and weight loss. Most patients have generalized lymphadenopathy, and about 40 per cent have skin lesions with maculopapular erythema, purpura, urticaria, or exfoliative erythroderma. Lymph-node biopsy specimens demonstrate the most characteristic histopathologic features: extensive effacement of lymph nodal architecture; a pleomorphic population of immunoblasts, plasma cells, lymphocytes, and eosinophils; interstitial deposits of eosinophilic material; and prominent vascular proliferation, with "arborization" of small vessels. The pathogenesis of angioimmunoblastic lymphadenopathy is still unknown, but its histopathologic features and laboratory findings strongly suggest that it is an immunologically mediated disorder. Some clinical and laboratory evidence supports the possibility that angioimmunoblastic lymphadenopathy is a benign reactive or proliferative process, whereas other studies suggest that it might be a malignant disease. In some patients, it can develop into immunoblastic sarcoma or other types of malignant lymphoma or leukemia. It is probably reasonable to consider angioimmunoblastic lymphadenopathy a prelymphomatous state of immunoblastic sarcoma.
 ...
PMID:Angioimmunoblastic lymphadenopathy. 391 79

Fifty-seven patients with refractory acute leukemia were treated with high-dose cytosine arabinoside to establish the maximum tolerated dose and duration and to determine the antileukemic activity. The maximum tolerated regimen was found to be 3 g/sq m every 12 hr for 6 days. At this dose, nonhematologic toxicity was limited to conjunctivitis in approximately half of the patients, and liver toxicity (transient elevations in transaminase, alkaline phosphatase, or bilirubin) was frequently observed, but neither was dose-limiting. Extending the duration of treatment to 8 days resulted in excessive diarrhea and skin toxicity (painful erythema with bullae), while increasing the dose to 4.5 g/sq m q. 12 hr for 6 days resulted in severe cerebellar toxicity. Myelosuppression was severe, but was not related to the intensity of treatment; granulocyte recovery occurred a median of 28 days (range 22-40 days) after initiating therapy, and platelet recovery occurred after a median of 25 days (range 16-41 days). Antileukemic activity was evaluable in the 46 patients who survived at least 3 wk. Complete remissions were obtained in 1 of 6 patients with chronic myelogenous leukemia (CML) in accelerated phase and 1 of 3 acute lymphoblastic leukemia (ALL) patients. A more detailed analysis of response was possible for the 37 evaluable patients with acute nonlymphoblastic leukemia: 70% of these patients responded, with 51% complete remissions. The median unmaintained response was 4 mo (range 2-26+ mo). The complete response rate was higher in patients who received at least 12 doses of high-dose cytosine arabinoside compared to shorter regimens [17/28 (61%) versus 2/9 (22%), p less than 0.05]. Resistance to cytosine arabinoside in conventional doses was documented in 11 patients, 5 of whom responded (2 complete remissions) to high-dose regimens. We conclude that high-dose cytosine arabinoside in the maximally tolerated regimen of 3 g/sq m every 12 hr for 6 days has substantial antileukemic activity in patients refractory to standard therapy. Durable unmaintained remissions can be achieved, even in patients who fail to respond to cytosine arabinoside in conventional doses.
 ...
PMID:High-dose cytosine arabinoside therapy for refractory leukemia. 622 74

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly, but flared up after several years. Skin lesions appeared in the form of erythema, papules, or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was 0%-2%. The serum lactate dehydrogenase (LDH) value was within normal range and was not associated with hypercalcemia; lymphadenopathy, hepatosplenomegaly, and bone marrow infiltration were very slight. In most cases, hypergammaglobulinemia was seen, and in one case, monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their anti-ATLA antibodies were positive; none had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness and died of cryptococcus meningitis. Based on clinical and pathologic differences, we believe that these cases should be distinguished from typical ATL cases for the purposes of prognosis and treatment.
 ...
PMID:A proposal for smoldering adult T-cell leukemia: a clinicopathologic study of five cases. 622 22


1 2 3 4 5 6 7 8 Next >>