11/67. Multifocal progressive leukoencephalopathy occurring after refractory anemia and multiple infectious disorders consecutive to severe lymphopenia.Progressive multifocal leukoencephalopathy (PML) is related to central nervous system infection with jc virus (JCV). This leukoencephalopathy occurs in immunocompromised patients such as those with acquired immunodeficiency syndrome (AIDS) or lymphoid malignancies. We describe here a patient with myelodysplastic syndrome who developed several life-threatening infections including listeriosis, tuberculosis, and PML. listeriosis and recurrence of tuberculosis preceded the occurrence of PML. Neurologic features associated with major ataxia, speech disorders, and PML were documented by cranial magnetic resonance imaging showing typical features in the cerebellum and proven by polymerase chain reaction (PCR) detection of JCV dna in the cerebrospinal fluid. No specific treatment was decided because of progression toward acute myeloid leukemia. In this case, PML occurred with no susceptibility and without immunosuppressive treatment. Our case adds further support to the association between the impairment of T-cell immune responses and myelodysplastic disorders.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
12/67. Unexpected toxicity after low-dose docetaxel treatment of a cancer patient with clinically latent HCV-positive hepatic cirrhosis.We report a case with unexpected toxicity after low-dose docetaxel chemotherapy. The patient had a history of clinically latent HCV-positive hepatic cirrhosis when she presented with inoperable pulmonary adenocarcinoma. She was recruited in a protocol combining standard radiotherapy (RT) with docetaxel (30 mg/m2 week). On day 7, after the 1st docetaxel infusion, grade III neutropenia (980 neutrophils), grade II platelet toxicity (90,000/ml) and lymphopenia (486/ml) had developed. Chemotherapy and RT were interrupted and the neutrophil counts were partially restored (1400/ml), while the platelet counts were back to normal (140,000/ml) and the lymphocyte counts were further reduced (320/ml), on day 15. Bilateral leg oedema and hair loss appreared. On day 21, there was a full restoration of neutrophil counts (1890/ml), while there was persistent lymphocytopenia (300/ml). alopecia grade III was now evident. Dysphagia grade II complicated with fungal oropharyngeal infection appeared on day 24 (24 Gy of RT). One more dose of docetaxel of 30 mg/m2 was given on day 36 Grade II neutropenia (1050/ml) and grade III platelet toxicity (48,000/ml) were observed 14 days after the second docetaxel dose, while dysphagia grade II appeared once again. After a one-week delay, RT was continued to a total dose of 54 Gy. liver function tests remained unchanged throughout the treatment. Post-RT CT-scan of the chest and upper abdomen showed complete response of the lung lesion. We suggest that, when docetaxel is chosen to treat cancer patients with HCV-positive hepatic cirrhosis, a starting dose schedule reduced by at least 50% should be considered before escalating to the standard dose.- - - - - - - - - - ranking = 0.5keywords = infection (Clic here for more details about this article) |
13/67. Pure red cell aplasia due to parvovirus following treatment with CHOP and rituximab for B-cell lymphoma.A 26-year-old woman, diagnosed with diffuse large B-cell lymphoma, was treated with CHOP (cyclophosphamide, hydroxydaunomycin, oncovin, prednisone), rituximab and radiotherapy. She developed transfusion-dependant anaemia, which persisted following chemotherapy. bone marrow aspirate and biopsy were consistent with pure red cell aplasia and parvovirus infection. serology was negative for previous or acute infection but parvovirus dna was detected by polymerase chain reaction. Administration of intravenous immunoglobulin (1 g/kg) resulted in reticulocytosis and recovery of her haemoglobin. We hypothesize that rituximab caused depletion of her normal B cells, resulting in an inability to mount a primary immune response to parvovirus infection.- - - - - - - - - - ranking = 1.5keywords = infection (Clic here for more details about this article) |
14/67. Transient panhypogammaglobulinaemia and B-lymphocyte deficiency in a patient with neuropsychiatric systemic lupus erythematosus after immunosuppressive therapy.Panhypogammaglobulinaemia is a rare complication of systemic lupus erythematosus (SLE), but its cause and mechanism are unclear. We observed transient panhypogammaglobulinaemia in a patient with neuropsychiatric SLE after treatment with prednisolone and cyclophosphamide. After the patient developed recurrent infections, laboratory findings disclosed panhypogammaglobulinaemia with B-lymphocyte deficiency. The serum immunoglobulin level returned to the normal range after the prednisolone was tapered off. Because lupus patients are susceptible to infections associated with disease exacerbation or immunosuppressive treatment, recurrent infections might be expected during the disease course without need for further evaluation of the immunodeficiency. However, this reversible, probably drug-induced case of hypogammaglobulinaemia highlights the need for immunoglobulin measurements when immunodeficiency is suspected in lupus patients.- - - - - - - - - - ranking = 1.5keywords = infection (Clic here for more details about this article) |
15/67. mycoplasma pneumoniae-associated stevens-johnson syndrome exhibits lymphopenia and redistribution of CD4 T cells.erythema multiforme (EM) is an immune-mediated disease categorized into EM minor and EM major, also called stevens-johnson syndrome. The presence of mucosal involvement differentiates erythema multiforme major from erythema multiforme minor. Many drugs and agents can induce stevens-johnson syndrome. We report a case of stevens-johnson syndrome associated with mycoplasma pneumoniae infection. lymphopenia with a significant decrease of CD4 T cells in the blood and predominant CD4 T cells in the skin vesicular fluid was found. The improvement of lymphopenia was associated with disease recovery. In a retrospective chart review of patients treated in our hospital over the past 3 years, we found that 5 patients with stevens-johnson syndrome all had lymphopenia (< 1.50 x 10(9)/L; average 0.99 x 10(9)/L), whereas 13 other patients with erythema multiforme minor demonstrated normal lymphocyte counts (average 3.13 x 10(9)/L), with the exception of one patient with herpes infection showing lymphopenia. These results suggested that an immunopathogenesis involving redistribution of CD4 T cells might contribute to the development of stevens-johnson syndrome. Further studies to investigate the involvement of CD4 T cells in stevens-johnson syndrome may implicate a specific strategy to prevent fatal stevens-johnson syndrome.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
16/67. patients with systemic lupus erythematosus at risk for pneumocystis carinii pneumonia.We describe 6 cases of patients with systemic lupus erythematosus (SLE) who developed pneumocystis carinii pneumonia. All were treated with high dose corticosteroids, and all developed the infection within 4 months of beginning new or revised cytotoxic therapy. All patients tested (5 of 6) were negative for human immunodeficiency virus (hiv). Those patients who developed pneumocystis carinii pneumonia had more severe lymphocytopenia (median 595 vs 833/mm3) and received higher doses of corticosteroids (median prednisone dose = 43 vs 20 mg/day) than other patients with active SLE. A threshold lymphocyte count of 350/mm3 identified 4 of 6 cases but only 1 of 20 controls. patients with SLE treated with high dose corticosteroids and cytotoxic drugs and with severe lymphocytopenia may be at increased risk for this opportunistic infection.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
17/67. Reversible dysfunction of t-lymphocytes in common variable immunodeficiency.A 30-year-old man with recurrent sinopulmonary infections, eventually fatal, was found to have common variable immunodeficiency. In addition to low serum immunoglobulin concentrations he also had lymphopenia and cell-mediated immunodeficiency as shown by cutaneous anergy and a poor lymphocyte response to phytohemagglutinin (PHA) in vitro. However, intradermal injection of PHA produced a vigorous cutaneous response, showing that some cell-mediated responsiveness remained. The responsiveness of his lymphocytes to PHA was restored towards normal (confirmed by chromosome studies) by the addition of a small number of normal leukocytes to cultures; thus a reversible functional defect in his t-lymphocytes was revealed. Experiments indicated that the defect was cellular and not due to serum factors and it was concluded that normal leukocytes restored a missing factor to the patient's t-lymphocytes. Although counts of macrophage precursor cells in the bloodstream were low, thus contributing to the immunodeficiency, this could not have caused the reduced PHA response. Several relatives of this patient had lymphoma; two cousins had common variable immunodeficiency.- - - - - - - - - - ranking = 0.5keywords = infection (Clic here for more details about this article) |
18/67. Recurrent 'sterile' verrucous cyst abscesses and epidermodysplasia verruciformis-like eruption associated with idiopathic CD4 lymphopenia.rupture of follicular (epidermoid) cysts is believed to be the consequence of bacterial infection. We report a 24-year-old man with idiopathic CD4 lymphopenia and chronic mycobacterium avium intracellulare infection who developed multiple, recurring painful abscesses over the distal extremities that increased in number and severity when systemic steroid and interferon-gamma treatment was instituted for interstitial lung disease. Cultures were consistently negative for microorganisms, but pathological examination revealed ruptured epidermoid cyst walls with human papillomavirus (HPV) viropathic changes (keratinocytes with perinuclear halos and abundant basophilic keratohyaline granules). Cutaneous examination showed numerous, widespread flat-topped papules and achromic macules over the extremities, head and neck. Nested polymerase chain reaction analysis for HPV dna revealed that the abscess-related cyst walls harboured epidermodysplasia verruciformis (EV)-associated HPV types 20, 24, alb-7 (AY013872) and 80. His cutaneous lesions harboured HPV types 3, 8 and 80. Similar to past reports, our patient developed an EV-like eruption in the setting of immunodeficiency. In this instance, EV-associated HPV infection of the follicular infundibular epithelium or pre-existing cysts in the setting of immunodeficiency may have led to cystic growth, rupture and subsequent painful inflammation.- - - - - - - - - - ranking = 1.5keywords = infection (Clic here for more details about this article) |
19/67. failure to thrive in a 14-month-old boy with lymphopenia and eosinophilia.We describe the case of a 14-month-old boy with delayed-onset SCID due to ADA-deficiency which was masqueraded only by failure to thrive. Remarkably, the child had no serious infections and an adequate immune response. However, absolute lymphopenia, eosinophilia and absent thymus on chest x-ray were indicative for immunodeficiency.- - - - - - - - - - ranking = 0.5keywords = infection (Clic here for more details about this article) |
20/67. A 55-year-old man with hypogammaglobulinemia, lymphopenia, and unrelenting cutaneous warts.A 55-year-old white man with a history of hypertension, fibromyalgia, and colonic polyps presented with unrelenting plantar warts on his hands and feet for the past 4 years. He was otherwise healthy and without a history of recurrent infections. physical examination was unremarkable except for extensive warts on his hands and feet. Pertinent laboratory findings included hypoalbuminemia, hypogammaglobulinemia, and lymphopenia most severely affecting CD4( ) T cells. Testing for hiv infection was negative. This clinical and laboratory presentation suggested a combined humoral and cellular immunodeficiency syndrome that could be best explained by loss of lymphocytes, immunoglobulins, and other serum proteins. Additional immunologic testing revealed a marked reduction in peripheral blood naive (CD4( )CD45RA( )) T cells. A 24-hour stool collection showed a markedly elevated alpha(1)-antitrypsin level. These findings were most consistent with the diagnosis of intestinal lymphangiectasia, a type of protein-losing enteropathy associated with hypoalbuminemia, hypogammaglobulinemia, and lymphopenia, characterized by a preferential loss of naive CD4( ) T cells into the gastrointestinal tract. This case illustrates the importance of considering intestinal loss of immunoglobulins and lymphocytes in the differential diagnosis of the adult patient who presents with laboratory evidence of a combined humoral and cellular immunodeficiency. It also underscores the diagnostic utility of the clinical immunology laboratory and how flow cytometry, in particular, can contribute to an understanding of pathogenic mechanisms.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
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