Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 13-year-old boy who had undergone allogenic bone marrow transplantation for treatment of acute lymphocytic leukemia presented with bilateral periorbital rash and swelling. A CT scan showed bilateral symmetric periorbital swelling, subconjunctival fluid collections, and lacrimal gland enlargement. The patient was initially treated for presumed cellulitis. However, persistently negative regional cultures (eye, nasopharynx), a rapid response to immunosuppressive therapy after several days of nonresponse to intravenous antibiotic therapy, and ultimately, results of a skin biopsy confirmed the diagnosis of acute graft-versus-host disease.
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PMID:CT demonstration of periorbital graft-versus-host disease. 912 39

A prospective study on the microbes isolated from the alimentary tract in 120 bone marrow transplant (BMT) recipients (1991-1993) was undertaken to define the spectrum of organisms isolated under antimicrobial prophylaxis, their temporal sequence of emergence, and the associated morbidity and mortality. Clostridium difficile (n = 20), isolated in the pre-engraftment and early post-engraftment periods (day 2-45 post-BMT), was the most common microbe recovered from stool of patients with diarrhea. In contrast to previous reports, no significant difference in mortality was observed between patients with and without C. difficile isolated in stool. Two patients had neutropenic ileocecitis with concomitant bacteremia due to Escherichia coli and Klebsiella pneumoniae. One patient was found to have astrovirus gastroenteritis (day 7), and Giardia lamblia was recovered from the stool of another (day -7). Heavy growth of Staphylococcus aureus from direct smear-positive specimens was found from the upper airway of two patients with severe mucositis and complete dysphagia (day 12 and 23). Salmonella spp. of groups B and E were found in the stool of five asymptomatic patients at the time of conditioning. No specific organisms was recovered from the endoscopic brushing of two patients with lower end esophagitis, three patients with upper gastrointestinal bleeding, and three patients with perirectal cellulitis. During the post-engraftment period, five patients had documented cytomegalovirus gastroenterocolitis (days 34-97), one had Mycobacterium chelonae colitis (day 70), and another had nodular gastritis due to Acremonium falciforme (day 270). Overall, only 28% of patients with alimentary tract symptoms/syndrome had specific pathogens isolated from clinical specimens. Differentiation of the causation of alimentary tract symptoms was often difficult because noninfectious complications such as conditioning toxicity, graft-versus-host disease, and its treatment often caused alimentary tract symptoms in addition to predisposed BMT patient to infection. The reluctance of obtaining tissue biopsy for ascertaining the importance of those potential alimentary tract pathogens often dictate the use of empirical treatment.
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PMID:Clinical significance of alimentary tract microbes in bone marrow transplant recipients. 955 72

Fusarium species frequently implicated in human infections include F. solani, F. oxysporum and F. moniliforme. Among immunocompetent patients, tissue breakdown (as caused by trauma, severe burns or foreign body) is the risk factor for fusariosis. Infections include keratitis, onychomycosis and occasionally peritonitis and cellulitis. Treatment is usually successful and requires removal of the foreign body as well as antifungal therapy. Among immunocompromised patients, mainly patients with haematological malignancies, Fusarium spp. are the second most common pathogenic mould. Risk factors for disseminated fusariosis include severe immunosuppression (neutropenia, lymphopenia, graft-versus-host disease, corticosteroids), colonisation, tissue damage, and receipt of a graft from an HLA-mismatched or unrelated donor. Clinical presentation includes refractory fever (> 90%), skin lesions and sino-pulmonary infections ( approximately 75%). Type of skin lesions includes ecthyma-like, target, and multiple subcutaneous nodules. Skin lesions lead to diagnosis in > 50% of patients and precede fungemia by approximately 5 days. In contrast to disseminated aspergillosis, disseminated fusariosis can be diagnosed by blood cultures in 40% of patients. Histopathology reveals hyaline acute-branching septate hyphae similar to those found in aspergillosis. Mortality from fusarial infections in immunocompromised patients ranges from 50% to 80%. Host immune status is the single most important factor predicting outcome. Persistent neutropenia and corticosteroid therapy significantly affect survival. Optimal treatment has not been established. Anecdotal successes have been reported with various agents (high-dose amphotericin B, lipid-based amphotericin B formulations, itraconazole, voriconazole) and with cytokine-stimulated granulocyte transfusions. Preventing fusariosis relies on detection and treatment of cutaneous damage prior to commencing immunosuppression and decreasing environmental exposure to Fusaria (via air and water).
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PMID:Human fusariosis. 1474 3

We describe the case of a 38-year-old male patient who had acute myeloid leukemia and developed prolonged neutropenia after induction chemotherapy. He developed thrombotic complications at multiple sites. Thrombophlebitis of the hemorrhoidal plexus became exacerbated and developed into critical cellulitis. Because the patient had no human leukocyte antigen-identical sibling, we considered an alternative donor. Because of the necessity for early neutrophil recovery to resolve the critical infection, we proceeded with allogeneic peripheral blood stem cell transplantation (PBSCT) from a microchimeric haploidentical sibling donor. We infused peripheral blood mononuclear cells directly into the patient without cryopreservation and thawing procedures. We aimed for the contaminating granulocytes to act as a granulocyte transfusion. Actually, the neutrophils increased to 1.6 x 10(9)/L on day 1, when the patient showed a temporary resolution of infection. Engraftment was achieved shortly after neutropenic nadir, and acute graft-versus-host disease (GVHD) has been well controlled. Although the patient experiences extensive chronic GVHD, he has been well as an outpatient with a 90% Karnofsky performance status score. The leukemia has been in complete remission for more than 1 year. These findings suggest the clinical utility of a salvage therapy with allogeneic PBSCT from a microchimeric haploidentical donor to treat refractory leukemia concurrent with life-threatening infection.
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PMID:Successful stem cell transplantation without cryopreservation from a microchimeric haploidentical sibling for refractory acute myeloid leukemia complicated with critical thrombophlebitis and cellulitis. 1726 7

There are numerous dermatoses which may cause cicatricial alopecia when localized on the scalp, such as chronic discoid lupus erythematosus (DLE), lichen planus, graft-versus-host disease, dermatomyositis, scleroderma, cicatricial pemphigoid, porphyria cutanea tarda, follicular mucinosis, perifolliculitis capitis abscedens, lichen sclerosus et atrophicus, necrobiosis lipoidica, sarcoidosis, etc. Histologically, cicatricial alopecia is characterized by dermal scarring, along with absent or reduced hair follicles and reduced number of erector pili muscles. According to working classification of cicatricial alopecia by the North American Hair Society, primary cicatricial alopecia may be divided into the following categories: lymphocytic group (e.g., DLE, lichen planopilaris, classic pseudopelade (Brocq), central centrifugal cicatricial alopecia); neutrophilic group (e.g., folliculitis decalvans, dissecting cellulitis); and mixed group (e.g., folliculitis keloidalis). Over a 5-year period, 36 patients with cicatricial alopecia were hospitalized at our Department: DLE (n = 27), pseudopelade Brocq (n = 3), mucinosis follicularis (n = 2), and lichen planopilaris, folliculitis decalvans, folliculitis abscedens and folliculitis keloidalis (one patient each). Clinical evaluation was compared with histopathologic analysis of follicular architecture, as well as with the type, localization and extent of inflammatory infiltrate. Scalp biopsy was considered mandatory in all cases. Our experience indicates the need of more complex research to extend the knowledge about the etiopathogenesis and treatment options for cicatricial alopecia. We hope that this type of alopecia may attract more attention and research in the future.
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PMID:Cicatricial alopecia as a manifestation of different dermatoses. 1731 39

Leukocyte adhesion deficiency type I (LAD-I) is an inherited immunodeficiency disorder caused by defective expression of the leukocyte integrins, namely, lymphocyte function-associated antigen 1, Mac-1, and p150, 95, and is associated with obstructed cell adhesion, migration, and phagocytosis. Patients suffer from various bacterial or fungal infections and their prognoses are poor. The only curative treatment is hematopoietic stem cell transplantation. Conventional myeloablative transplantations have been performed, but with unsatisfactory results. We performed the first successful nonmyeloablative unrelated marrow transplantation for a 20-year-old female LAD-I patient, who suffered from recurrent and occasionally life-threatening infections such as cellulitis, gingivostomatitis, and sepsis. We adopted a preparative regimen with fludarabine, cyclophosphamide, and low-dose total-body irradiation, and tacrolimus and short-term methotrexate as immunosuppressants. This procedure was sufficiently immunosuppressive to obtain stable engraftment without remarkable complications, and graft-versus-host disease was controllable. Dramatic improvement of her disease was observed, supported by the normal expressions of integrins. Twenty one months after transplantation, she is well with a Karnofsky score of 100. Thus, nonmyeloablative transplantation is considered a feasible method for LAD-I.
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PMID:Successful nonmyeloablative bone marrow transplantation for leukocyte adhesion deficiency type I from an unrelated donor. 1767 74