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Successful interferon-gamma therapy in a chronic granulomatous disease (CGD) patient suffering from Staphylococcus aureus hepatic abscess and invasive Candida albicans infection.

A 16-year old boy with an early history of recurrent lower respiratory tract infections exhibited symptoms of prolonged septic fever and liver abscess. Cultures from liver puncture were positive for Staphylococcus aureus, and the patient initially responded to antibacterial therapy. After a period of 4 months, however, the infection relapsed, and further treatment with broad antibacterial, antifungal and tuberculostatic drugs was ineffective. Neither soluble nor particulate stimuli were found to elicit the respiratory burst response in granulocytes from the patient. Spectral analysis of granulocyte cytochrome-b confirmed the diagnosis of chronic granulomatous disease. Since the patient's physical condition deteriorated severely during the prolonged (10-week) septic course, immunosupportive interferon-gamma was added to the anti-microbial therapy. With this regime, the fever subsided and the general condition of the patient improved dramatically. He could be discharged from hospital 9 weeks after the introduction of interferon-gamma and was, at an elective follow-up control 1 month later, convalescing and showed no signs of active infection.

Adolescent↗

[Second marrow transplantation following high dose busulfan, etoposide, and Ara-C after testicular relapse in a patient with AML].

Prognosis of second marrow transplantation after leukemia relapse is usually gloomy. We report a patient with AML who was successfully treated by the second marrow transplant following high dose busulfan, etoposide, and Ara-C for the testicular relapse after the first marrow transplantation. A 24-year-old man was diagnosed as having acute myeloid leukemia (AML) in September, 1988. In December of 1989 when he was in early relapse after his 2nd remission, he received the first allogeneic BMT from his HLA identical brother after high dose busulfan and cyclophosphamide conditioning. His posttransplant course was uneventful and graft versus host disease was not observed. Three months after BMT, he noticed swelling on right testicle. Leukemic cell infiltration was confirmed by aspiration cytology. The testicular relapse was followed by marrow relapse. After successful remission induction chemotherapy, he received 17.5 Gy testicular irradiation and second marrow transplantation using high dose busulfan, etoposide, and Ara-C conditioning. Although his posttransplant period was complicated by severe mucositis, high fever and bronchopneumonia, hematologic recovery was obtained by 3 weeks after the second transplant. He is now continuing in complete remission 18 months after the second BMT. This case report suggests that the combination of high dose busulfan, etoposide, and Ara-C could be a choice as a conditioning regimen for resistant AML relapsing after BMT.

Adult↗

[A recovered case of SLE with central nervous system involvement who relapsed presenting new symptoms associated with development of serum anti-Sm antibody].

A rare SLE patient with central nervous system involvement (CNS-SLE) who relapsed presenting new symptoms associated with the development of serum anti-Sm antibody and was then successfully treated with cyclophosphamide (CY) pulse therapy is presented here. A 47-years old housewife was admitted to Kushiro City General Hospital because of fever, limb erythema and drowsy consciousness in September 1995. On the basis of convulsion, proteinuria, leukopenia, thrombopenia, serum positive tests for both anti-nuclear antibody and anti-SSA antibody and low complement levels, as well as elevations of IgG index and IL-6 in the cerebrospinal fluid (CSF), she was diagnosed as having CNS-SLE. Serum tests for anti CL-beta 2 GPI antibody and lupus anticoaglant was negative. Serum test for HBs antigen was positive. She was treated successfully with methylprednisolone (mPSL) pulse therapy and plasma exchange (PE). Prednisolone was gradually tapered to the dosage of 17.5 mg per day and she was discharged in April 1996. She was re-admitted because of fever, an exacerbation of skin eruption and arthralgia in October 1996. Serum anti-Sm antibody was found to be positive. mPSL pulse therapy was not effective. On the basis of hallucination and elevations of IgG index and IL-6 in the CSF, a diagnosis of relapsed CNS-SLE was made. However the level of IFN-alpha in the CSF was normal. Although PE was not effective, CY pulse therapy was markedly effective.

Autoantibodies↗

[Successful meropenem therapy of recurrent multiple brain abscess].

Authors describe the history of a 37-year-old man suffering from multiple purulent brain abscess. The multiple brain abscess evolved primarily from a gluteal abscess to the lung, and secondarily from the lung to the brain by hematogenous spreading of the bacteria. The identification of the pathogene/s was unsuccessful despite numerous bacteriological examination. Despite many regimens of empiric antibacterial therapy the brain abscesses progressed, neurologic state of the patient deteriorated. At long last, the patient was given chloramphenicol. After that, he had no more fever, his consciousness cleared, no more epileptic convulsion occurred and the cell number of the cerebrospinal fluid became normal. The patient was thought to be cured and was sent home. Two months later fever occurred again and it was accompanied by excrutiating headache, increasing disorientation, so the patient was admitted to the hospital. The occurrence of a new brain abscess and purulent meningitis indicated the relapse of the disease. It was again unsuccessful to identify the pathogene/s therefore the authors treated the patient with many empiric antibiotic regimen, all of which-including chloramphenicol too--proved to be uneffective. As all the therapeutic regimens usually used in the treatment of purulent brain abscess were uneffective--including the combinations which have the widest antibacterial spectrum, authors gave meropenem as ultimum refugium. Some days later the fever came to an end, his consciousness cleared, the brain pressure and the cerebrospinal fluid became normal. The patient had no serious complaints in the course of the four years follow up, his residual neurologic symptoms regressed. On the base of this case history, authors suppose that meropenem--which has already proved to have a very wide antibacterial spectrum and to be very effective in the therapy of many kinds of serious bacterial infections--could also become a promising new therapeutic alternative in the treatment of purulent brain abscess.

Adult↗

Nitrogen mustard, vincristine, procarbazine, and prednisolone for relapse after radiation in Hodgkin's disease. An analysis of long-term follow-up.

One hundred and sixty-one patients who were treated with nitrogen mustard, vincristine, procarbazine, and prednisolone (MOPP) chemotherapy for Hodgkin's disease have been observed for a median of 10.2 years. Eighty-two percent of those patients received MOPP after relapse from previous irradiation. The complete response (CR) rate was 71%. For the 116 patients achieving CR the relapse-free survival at 5 years was 83% and at 10 years, 79%. The overall survival was 72% at 5 years and 64% at 10 years. In a stepwise logistic regression analysis the most important clinical factors influencing response were B symptoms at presentation (fever greater than 38 degrees C, night sweats, weight loss greater than 10% of body weight), histologic subtype, and lung or pleural involvement. Patients who received MOPP as first-line therapy had a significantly worse response rate than those who received MOPP upon relapse after radiotherapy. This difference is reduced when adjustments are made for the presence of the above prognostic factors. A Cox regression analysis showed that Stage IV at presentation and lymphocyte-depleted histology were the most important factors indicating reduced survival. Patients who achieved a CR to MOPP had a significantly improved survival. Of the 65 patients who had died at the time of the analysis of this series, 46 died of progressive Hodgkin's disease. All four patients who developed secondary acute nonlymphocytic leukemia had received radiation as well as MOPP.

Adolescent↗

About five cases of acute rheumatic fever in the adult.

We describe five cases of acute rheumatic fever in adults (applying Jones's criteria and exclusion of other common causes of acute polyarthritis in adults). The polyarthritis was migratory in three patients and additive in two. Severe tenosynovitis was present in three patients. Only one patient had carditis and received corticoids. The others improved with aspirin. The disease subsided after a time period varying from one week to two months and did not relapse.

Adult↗

Acute myelo-monocytic infiltrate of the lower esophagus in a 4-year-old renal transplant recipient.

BACKGROUND: Acute myeloid leukemia after solid organ transplantation is a rare phenomenon. Few achieve remission; most succumb to relapse and infection. METHODS: A 4-year-old male renal transplant recipient on triple immunosuppression had culture-negative high spiking fever, persistent leukopenia, anemia and severe gastritis. Upper endoscopy showed 2 ulcerating masses in the lower esophagus. RESULTS: Esophageal biopsy showed a highly atypical myelo-monocytic infiltrate. A blastic population of cells featuring convoluted nuclear envelopes with an open chromatin pattern and abundant cytoplasm were filling the submucosa and infiltrating into the muscularis propria. Extensive investigation including bone marrow aspiration showed no spread. Drastic reduction of immunosuppression except 4-mg/d (0.22 mg/kg/d) prednisone for 2 to 3 weeks led to resolution of the leukemic process proven on repeat biopsy. The patient still is in remission 2 years later. CONCLUSION: This case provides evidence that early diagnosis and aggressive reduction of immunosuppression may remit a posttransplant locally invasive acute myelo-monocytic infiltrative process.

Biopsy↗

Cytomegalovirus-associated manifestations involving the digestive tract in children with human immunodeficiency virus infection.

OBJECTIVE: To study the clinical manifestations of gastrointestinal cytomegalovirus disease in children with human immunodeficiency virus infection. METHODS: Review of clinical records of eight human immunodeficiency virus-infected children and histopathologically confirmed gastrointestinal cytomegalovirus disease from 1995 to 2001. RESULTS: Six of the eight children were younger than 1 year. The most common clinical presentations were fever and chronic diarrhea. Lower gastrointestinal hemorrhage and bowel perforation were noted in four and three patients, respectively. The colon was the most commonly affected site, followed by the small bowel and esophagus. The diagnosis was established by histopathology, obtained during endoscopy and surgery. Mucosal edema, erythema, and ulcer comprised the most common endoscopic findings. Two patients with fever, chronic diarrhea, and lower gastrointestinal bleeding developed remission after being treated with a 14-day course of ganciclovir. CONCLUSION: Gastrointestinal cytomegalovirus disease can result in serious life-threatening complications, such as bowel perforation and massive gastrointestinal bleeding. Patients with chronic diarrhea and fever of unidentified cause might benefit from gastrointestinal endoscopy for early diagnosis and treatment. Although ganciclovir does not eradicate the infection and relapses are frequent, this treatment can prevent complications and reduce morbidity.

AIDS-Related Opportunistic Infections↗

Organizing pneumonia-like process: an unusual observation in steroid responsive cases with features of chronic interstitial pneumonia.

Two patients under long-term surveillance showed the similar clinical features of low-grade fever, scanty productive cough, progressive dyspnea, and roentgenologic findings of lung infiltrates. Both patients responded only to systemic corticosteroid therapy and suffered relapses when it was discontinued. In one patient, who was found to be IgA-deficient, the pulmonary disease followed an episode of subacute thyroiditis; in this patient the intake of cephalosporin and subsequent rechallenge with the drug aggravated the disease. Needle biopsies in both patients showed the features of organizing intra-alveolar pneumonia. The histopathologic findings and their relation to the clinical symptoms are discussed.

Adrenal Cortex Hormones↗

[Reactional status of leprosy].

Reactional leprosy is studied according to its clinical forms A) Lepromatous a) Acute lepromatization: encroaching and invasive nature; the patient becomes more and more lepromatous ; bad prognosis. b) Erythema nodosum: "contusiform dermatitis"; variable prognosis not so bad as it is in the preceding case; allergic nature and its evolution is usually detained and therapeutics efficient. c) Erythema multiform. d) Lucio's phenomenon: vascular lesions and consequently necrosis as a complication of the "erythema necrotisans" (beautiful leprosy). B) Tuberculoid Reactional tuberculoid is the only one in this benign type, the Mitsuda's test must always be positive and prognosis consequently good. C) Dimorphous or "Borderline" whose Mitsuda's test is mostly negative, sometimes positive, but not stable. The lesions may stimulate the tuberculoid leprids but they invade mucous membranes, are impregnated by pigmentation, may present the Unna's band, and other characteristics of the Lepromatous type. Are associated (fever, asthenia and emaciation). Prognosis not very good, because of the possibility of lepromatization, according to its tendency. Evolution slower and frequent relapses. Besides there are nodular lesions. Pathogeny 1) Perifocal allergic reaction (Jadassohn). Similar to epituberculosis and Herxheimer reaction. 2) Septicemia. Sensitized tissues inside or outside the lesions, are invaded by the bacilli and so the allergic reaction takes place. Even without culture resources, Mycobacterium leprae has been found in the blood by direct examination. 3) Autoimmunization (Waldenstrom, Matthews and Trantman, 1965). Based upon the similarity between both humoral syndromes, in leprosy reactions and collagenous, diseases, as to: hypergammaglobulins, hypercryoproteins, antigammaglobulins, serological reactions (Wassermann, Kahn, Kline, VDRL) positives, Antistreptolysin O, protein C reactive, antinuclear factors, latex and Wadler-Rose test positives (rheumatoid tests) lowering of complement. If leprosy reaction is like this, it should be the less agressive of the autoimmune diseases. a) Its eruptions are cyclic not of long standing duration, as a general rule. b) Its prognosis has been recognized as good, except lately, because of the use of corticoid therapy which has been fatal, in many cases. After some years the leprosy reaction cures spontaneously. Treatment (see article)

Erythema Multiforme↗

Etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine for the treatment of metastatic, high-risk gestational trophoblastic disease.

OBJECTIVE: To evaluate the efficacy and toxicity of a regimen of etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine in patients with metastatic, high-risk gestational trophoblastic tumors. METHODS: Twelve women with metastatic gestational choriocarcinoma received 64 treatment cycles. All met the National Cancer Institute criteria for high-risk gestational trophoblastic tumors. Response was evaluated by monitoring serial serum beta-hCG levels. Toxicity was recorded using standard World Health Organization criteria. RESULTS: There was no life-threatening toxicity. Neutropenia necessitating a 1-week delay of treatment occurred with only eight treatment cycles (12.5%) and deferral of vincristine and cyclophosphamide with three cycles. Anemia requiring transfusion complicated only two cycles. Peripheral neuropathy in two patients was treated by discontinuing vincristine. Other toxicities included nausea and vomiting, diarrhea, stomatitis, alopecia, conjunctivitis, thrombocytopenia, and fever. Ten of the 12 subjects experienced a complete response. Two had partial responses and one with an initial complete response had relapse 4 months after completing therapy; all three were successfully salvaged with cisplatin-based chemotherapy. Overall survival was 100%, and all 12 patients are disease-free with a median follow-up of 26 months. CONCLUSIONS: Chemotherapy with etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine is well tolerated and highly effective for metastatic, high-risk gestational trophoblastic disease.

Adult↗