Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten of 70 children (14%) with acute lymphoblastic leukemia developed severe interstitial pneumonitis within three weeks after induction of central nervous system prophylactic therapy. The clinical picture was characterized by fever, cough, progressive dyspnea, and hypoxemia with complete resolution in one to three weeks, except in one patient who died during the acute illness from respiratory failure. P. carinii organisms were found in the lung tissue of only one patient. The etiology of the pneumonitis in the other nine children was probably viral, acquired or activated during a period of lymphopenia and immunosuppression. The morbidity and potential mortality from the pneumonitis warrants early recognition by open lung biopsy and intensive supportive therapy.
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PMID:Acute respiratory illness in children with acute lymphoblastic leukemia. 30 Jul 96

The present report describes an infant with severe combined immunodeficiency and cartilage-hair hypoplasia whose lymphocytes responded to thymosin in vitro. Immunologic evaluation was undertaken at 4 1/2 months of age following a history of recurrent severe infection. Family history included three cousins who died in early infancy, one from streptococcal meningitis and pneumonia, one from generalized varicella, and another from reticuloendotheliosis. Quantitative immunoglobulins were markedly depressed: IgG 141, IgA 0, and IgM 24 mg/100 ml. There was an absolute lymphopenia, multiple skin tests were negative, and in vitro lymphocyte responses to mitogens and antigens were depressed. Spontaneous E rosette determinations were 21% compared with control values of 65.7%. Erythrocyte adenosine deaminase (ADA) activity was normal. The patient's E rosette formation increased in the presence of thymosin, fraction 5, reaching a maximum of 56% with a concentration of 500 mug thymosin. Blastogenic responses to phytohemagglutinin also increased in the presence of thymosin. Transplantation of 24-week fetal thymus in Millipore diffusion chambers and subsequently transplantation of 18-week fetal thymus by intraperitoneal injection was accomplished. E rosettes increased to 35-40% and blastogenic responses to mitogens increased. Eight days after the second transplant the patient underwent a mild graft vs. host reaction which subsided after 1 week and mitogen blastogenic responses again increased to 5-8 times previous values, but still well below control ranges. Repeated episodes of pulmonary infection ensued, cor pulmonale resulted, and the clinical course was relentlessly downhill with the patient expiring from respiratory failure 5 months after transplantation.
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PMID:Severe combined immunodeficiency with cartilage-hair hypoplasa: in vitro response to thymosin and attempted reconstitution. 99 98

We evaluated 191 consecutive adults with pulmonary tuberculosis in order to develop methods to determine which patients should be initially hospitalized. Using stepwise discriminant analysis, we found the six factors that were most strongly associated with an unfavorable short-term outcome (respiratory failure or death): lymphopenia, advanced age, concomitant smear-positive extrapulmonary tuberculosis, alcoholism, a high percentage of neutrophils on the differential white blood cell count, and lack of radiographic evidence of cavitation. We derived a scoring system incorporating these variables and separated patients into high- and low-risk groups. The system was prospectively validated by applying it to a separate group of 179 patients. Lymphocyte-transformation tests in 32 patients revealed an association between clinical markers of poor prognosis and minimal lymphocyte proliferation to a heat-killed strain of Mycobacterium tuberculosis.
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PMID:Predictors of short-term prognosis in patients with pulmonary tuberculosis. 340 93

Pulmonary diseases are important cause of morbidity and mortality in patients with PM/DM. Thirteen (27%) out of 49 PM/DM patients in the study had developed respiratory failure. Respiratory failure resulted form interstitial pneumonitis (i.p.) in 6, pulmonary infection in 2 and both in 5 patients with PM/DM. Respiratory failure was fatal in PM/DM patients with pulmonary diseases and eleven of the 13 patients expired. More importantly, 2 PM/DM patients with respiratory failure had responded to chemotherapy, if it was due to pulmonary infection. Accordingly, it is almost important to distinguish i.p. and infection for the cause of respiratory failure. However, plain chest X-ray as well as standard laboratory tests failed to differentiate i.p. and pulmonary infection. On the other hand, high resolution CT of the lungs, serum endotoxin and serum beta-D-glucan were found to be useful for the differentiation of these conditions associated with respiratory failure in PM/DM patients. And additionally low serum level of IgG and lymphopenia at the onset of respiratory failure may suggest that the patients may have pulmonary infection rather than i.p.
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PMID:[Respiratory failure in polymyositis and dermatomyositis: differential diagnosis between pulmonary infection and interstitial pneumonitis]. 964 42

Severe acute respiratory syndrome (SARS) is a serious respiratory illness caused by a novel human coronavirus. The disease is highly infectious and carries significant mortality and morbidity. There was a major outbreak of SARS in Guangdong, Taiwan, Beijing, Hong Kong and Toronto between March and June 2003. Common presenting features of SARS are high fever, chills, rigor, malaise, nonproductive cough, lymphopenia and pulmonary infiltrates, followed by rapidly progressive respiratory failure in some cases. We describe two patients with systemic lupus erythematosus (SLE) who presented with fever, systemic upset and pulmonary infiltrates between April and June, 2003. One patient was confirmed to have coronavirus pneumonia while the other had active SLE with lung involvement. Our cases illustrate the difficult diagnostic dilemma in the evaluation of febrile SLE patients during the SARS epidemic.
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PMID:Lupus pneumonitis or severe acute respiratory syndrome? 1535 29

Partial or total CD3 chain expression defects including CD3 gamma, epsilon, delta, and zeta chain are among the autosomally inherited SCID presenting with T-B+NK+ phenotype with lymphopenia. The clinical findings are generally severe in all except for CD3 gamma deficiency. Here we present a 10-month-old CD3 gamma deficient boy with IBD. The patient had suffered from intractable diarrhea, recurrent pulmonary infections and oral moniliasis since two months of age. Following the first allogeneic HSCT from his HLA-identical (6/6) sister after a reduced intensity regimen, a second transplantation was performed five months later. On day +19 after second transplantation, the CD3 TCR alpha/beta chain expression increased to 66% with development of full donor chimerism (98.6%). A significant improvement in diarrhea, perianal lesions, and rectal fistula was observed suggesting an improvement in inflammatory bowel disease. The patient died at home on day +50 with a sudden respiratory failure secondary to an undetermined infection. The case was interesting being the first reported case with SCID and inflammatory bowel disease who responded very well to HSCT by full recovery of intractable diarrhea, failure to thrive, laboratory findings, and improvement of fistula formation.
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PMID:Hematopoietic stem cell transplantation in a CD3 gamma-deficient infant with inflammatory bowel disease. 1848 19

A 22-year-old man was admitted to our hospital with fever, cough and dyspnea. His chest radiograph showed diffuse ground-glass attenuation in both lung fields. Arterial blood gas analysis showed hypoxemia (PaO2 28.7 Torr breathing room air) and he required mechanical ventilation within 6 hours after admission. Gomori methenamine silver (GMS) stain of the bronchoalveolar lavage (BAL) fluid smear showed round and indented organisms, and polymerase chain reaction revealed pneumocystis jirovecii in the BAL fluid. The HIV antibody was positive and peripheral blood CD4-positive lymphocytes decreased to 4.0%. Pneumocystis pneumonia complicated with acquired immunodeficiency syndrome (AIDS) was diagnosed. There was no four-fold rise in screen viral titers. We treated him with antibiotics, trimethoprim-sulfamethoxazole, ganciclovir, fos-fluconazole, steroid pulse therapy and sivelestat sodium hydrate. Respiratory failure was relieved within 5 days following treatment. The percentage of neutrophils in the BAL fluid was elevated (44.6%). Neutrophil elastase on admission was increased and improved to the normal range after treatment. Sivelestat sodium hydrate is an anti-neutrophil elastase inhibitor and may be one of the treatment options for acute respiratory failure due to pneumocystis pneumonia in AIDS patients.
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PMID:[Acute respiratory failure due to pneumocystis pneumonia successfully treated with combined use of sivelestat sodium hydrate]. 1859 91

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by neoplasm or interstitial pneumonia that shows a rapid progressive course both of them are associated with a poor prognosis. A 56-year-old woman with no medical history was referred to the department of medicine because of arthralgia with a remarkable weight loss. She also complained of rapidly progressive dyspnea, cough and photosensitivity. Physical examination on admission showed scaly erythema on the dorsum of the hands (Gottron sign) and periorbital edema with a purplish appearance (heliotropic rash), arthritis, but no muscle weakness. Auscultation of the chest identified audible fine crackles on the lower aspects of both lungs. Results of laboratory findings on admission revealed a lymphopenia. The serum creatine kinase and serum lactate dehydrogenase concentration were normal. IRM muscle and electromyography were normal. Antinuclear antibody was positive 1:80 and anti-Jo-1 antibody and other autoantibodies to specific antigens were all negative. High resolution computed tomographic chest scans also revealed diffuse ground-glass opacities in both lungs with basilar predominance. Arterial blood gas analysis revealed hypoxia and hypocapnia. LBA was not performed because of the deterioration of respiratory symptoms. There was no neoplasm associated. The diagnosis of ADM complicated with ADM rapidly progressive interstitial pneumonia was made. Despite of IV methylprednisolone pulse therapy (1g*day-1 for 3 days) and cyclophosphamide, she died by respiratory failure.
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PMID:[Interstitial pneumonia complicating amyopathic dermatomyositis: a case report]. 1999 56

Patients with acquired immune deficiency syndrome (AIDS) are susceptible to secondary malignant tumors. Among those malignancies, the increased incidence of germ cell tumor (GCT) in patients with AIDS has recently been documented in Western countries, while that is still rare in Japan. Here, we report a man patient with advanced GCT (seminoma) complicated with AIDS who was continuously treated with highly active antiretroviral therapy (HAART). A partial response was obtained after resection of the primary left testis and three courses of chemotherapy. During the clinical course, he contracted unexpected gastric bleeding that made it impossible to take HAART agents and prophylactic agents for opportunistic infection. Thereafter, he suffered from a severe pulmonary infection and consequently died of severe respiratory failure. The lymphopenia related to both chemotherapy and AIDS synergistically rendered this patient immunoincompetent and thus he suffered from this fatal pulmonary infection. The recent progress in AIDS treatment has been reported to prolong the survival of tumor-bearing AIDS patients, especially GCT-bearing AIDS patients. Because of the current increase in the number of AIDS patients in Japan, it is important to report the present case which indicated that careful chemotherapy against GCT with strict management of the immunoincompetence can provide a good prognosis for GCT-bearing AIDS patients.
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PMID:Germ cell tumor concomitant with acquired immune deficiency syndrome. 2000 6

A 34-year-old HIV-positive patient with ulcerative colitis was transferred to the authors' hospital because of progressive worsening of his general condition with intermittent fever, increasing lymphopenia, anemia, thrombopenia and neutropenia under anti-tumor necrosis factor-(TNF-)alpha therapy with infliximab. In spite of negative screening tests before initiation of infliximab therapy and intermittent tests during treatment, miliary tuberculosis was finally diagnosed and a tuberculostatic therapy was started. The patient's clinical condition worsened due to the development of a serious exudative necrotizing pancreatitis which was likely to be caused by the tuberculostatic treatment. Due to severe pulmonary infiltrates and pleural effusions with respiratory failure the patient finally passed away.
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PMID:[Serious course of a miliary tuberculosis in a 34-year-old patient with ulcerative colitis and HIV infection under concomitant therapy with infliximab]. 2045 57


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