Cases reported "Immune System Diseases"

Filter by keywords:



Filtering documents. Please wait...

1/11. Extranodal marginal zone B-cell lymphoma of the lacrimal gland associated with crystal-storing histiocytosis.

    OBJECTIVE: Crystal-storing histiocytosis (CSH) is a very rare immunoglobulin (Ig) deposition disorder that can be associated with B-cell neoplasms as well as some reactive Ig-secreting lymphoproliferative disorders. This article reports the clinical, histopathologic, and molecular biologic findings of CSH in association with an extranodal marginal zone lymphoma (EMZL) of the lacrimal gland. DESIGN: Interventional case report with clinicopathologic correlation. INTERVENTION: Treatment consisted of conjunctival and orbital biopsies, as well as low-dose radiation of the left orbit. methods: Histopathologic findings of the conjunctival and orbital biopsies were compared. Further, extensive immunohistochemistry, polymerase chain reaction (PCR) for the detection of Ig heavy chain (IgH) gene rearrangement, Gene Scan analysis, and dna sequencing were performed on all tissues. RESULTS: The tumor manifestations in the specimens demonstrated a similar morphologic and immunophenotypic characteristics consistent with the diagnosis of EMZL. Immunoglobulin H PCR and Gene Scan analysis showed B cells derived from the same clone. In association with the orbital EMZL were large accumulations histiocytes filled with refractile crystals, consistent with the diagnosis of CSH. CONCLUSIONS: The current case describes an EMZL of the lacrimal gland appearing 5 years after excision and low-dose radiation of a conjunctival lymphoma. The orbital recurrence was associated with surrounding CSH, a rare Ig storage disorder more often reported in patients with multiple myeloma or lymphoplasmacytic lymphoma or immunocytoma. Ophthalmic pathologists should be aware of the diagnosis when reviewing orbital biopsies, because difficulties may arise when the crystal-storing macrophages occupy more tissue space than the Ig-secreting cells or when they resemble cell types such as rhabdomyoblasts.
- - - - - - - - - -
ranking = 1
keywords = macrophage
(Clic here for more details about this article)

2/11. Chronic endophthalmitis mimicking an endothelial immune reaction after penetrating keratoplasty.

    We describe a patient with chronic inflammation after combined penetrating keratoplasty and cataract surgery. This condition has been considered an unusual endothelial immune reaction. Cytopathological examination of the aqueous humor showed abundant neutrophil granulocytes, a few macrophages, and sparse lymphocytes. The predominance of neutrophil granulocytes but no macrophages or lymphocytes, as found in cases of an endothelial immune reaction, was interpreted as evidence of chronic endophthalmitis. Cytopathological evaluation of aqueous humor can be a helpful tool for differentiating between an endothelial immune reaction and chronic endophthalmitis after combined PKP and cataract surgery.
- - - - - - - - - -
ranking = 2
keywords = macrophage
(Clic here for more details about this article)

3/11. Schimke immuno-osseous dysplasia: two cases.

    We report two patients with Schimke immuno-osseous dysplasia (SIOD). SIOD is characterised by growth retardation, renal failure, spondylo-epiphyseal dysplasia, specific phenotype and defective cellular immunity. These two children demonstrated a bone dysplasia with characteristic radiographic appearances. We postulate that SIOD should be considered in all cases of growth failure with an unclassifiable bone dysplasia. Repeated urine tests for proteinuria could be helpful in reaching the correct diagnosis.
- - - - - - - - - -
ranking = 0.00061505451817016
keywords = bone
(Clic here for more details about this article)

4/11. Characterization of immunodeficiency in a patient with growth hormone insensitivity secondary to a novel STAT5b gene mutation.

    STAT5 proteins are components of the common growth hormone and interleukin 2 family of cytokines' signaling pathway. Mutations in the STAT5b gene, described in 2 patients, lead to growth hormone insensitivity that resembles laron syndrome. Clinical immunodeficiency was also present, although immunologic defects have not been well characterized thus far. Here we describe a 16-year-old girl who suffered generalized eczema and recurrent infections of the skin and respiratory tract since birth. She also suffered severe chronic lung disease and multiple episodes of herpetic keratitis. Clinical features of congenital growth hormone deficiency were observed, such as persistently low growth rate, severely delayed bone age, and postnatal growth failure resulting from growth hormone resistance. This combined phenotype of growth hormone insensitivity and immunodeficiency was attributable to a homozygous C-->T transition that resulted in a nonsense mutation at codon 152 in exon 5 of the STAT5b gene. This novel mutation determined a complete absence of protein expression. The main immunologic findings were moderate T-cell lymphopenia (1274/mm3), normal CD4/CD8 ratio, and very low numbers of natural killer (18/mm3) and gammadelta T (5/mm3) cells. T cells presented a chronically hyperactivated phenotype. in vitro T-cell proliferation and interleukin 2 signaling were impaired. CD4 and CD25 regulatory T cells were significantly diminished, and they probably contributed to the signs of homeostatic mechanism deregulation found in this patient. This new case, in accordance with 2 previously reported cases, definitely demonstrates the significant role of the STAT5b protein in mediating growth hormone actions. Furthermore, the main immunologic findings bring about an explanation for the clinical immunodeficiency features and reveal for the first time the relevant role of STAT5b as a key protein for T-cell functions in humans.
- - - - - - - - - -
ranking = 0.00030752725908508
keywords = bone
(Clic here for more details about this article)

5/11. Treatment of immune thrombocytopenic purpura in homosexual men.

    Over the past 3 yr we have treated 6 homosexual men (age 22-55 yr) with immune thrombocytopenic purpura. 4 of the 6 have antibody to HTLV-III in their serum, 1 of these patients has the acquired immune deficiency syndrome (AIDS), 1 has AIDS-related-complex (ARC), and a 3rd has persistent generalised lymphadenopathy (PGL). The platelet count at presentation was between 2 and 35 X 10(9)/l and in each case a bone marrow confirmed active platelet production. Antiplatelet antibodies were demonstrated in 3 of 4 patients tested. 3 of the 6 patients showed a partial response to prednisolone, 2 showed little or no response and the 6th showed a good response. 2 patients received high dose i.v. immunoglobulin - 1 had an excellent response prior to splenectomy, the other showed no response. 5 of the 6 patients had a splenectomy. 3 had a lasting remission (12-27 months after splenectomy), 1 of these has HTLV-III antibodies; 1 had a remission lasting 1 yr, followed by fluctuating thrombocytopenia (21-130 X 10(9)/l) and 1 showed no response.
- - - - - - - - - -
ranking = 0.00030752725908508
keywords = bone
(Clic here for more details about this article)

6/11. Histopathological study of otitis media with effusion.

    Thirteen temporal bones with otitis media with effusion (OME) taken from ten patients were studied. The effusions in the tympanum were histologically classifed into serous and mucoid types. In the serous effusion, neutrophils were dominantly distributed and the mucosa and submucosa showed inflammatory signs. Few epithelial cells and lymphocytes were observed in the effusion of the mucoid type. eosinophils were rarely seen in the effusions of both types. The presence of inflammatory process was a common finding in OME, particularly with serous effusion. A case, with an immunologic disorder suspected, showed an extensive proliferation of mucosa and unusually thick liquid in the middle ear. This histological feature of the case did not resemble those of other ears with mucoid type of effusion.
- - - - - - - - - -
ranking = 0.00030752725908508
keywords = bone
(Clic here for more details about this article)

7/11. Immune-mediated complications in patients with myelodysplastic syndromes--clinical and cytogenetic features.

    It has been recognized in recent years that some patients with myelodysplastic syndromes (MDS) develop immune-mediated complications (IMC), but little is known about the correlations to MDS-specific disease features. In a retrospective study of 82 MDS patients, we identified 10 (12%) with IMC (group A) and compared them to the remaining 72 cases (group B). Group A consisted of 5 patients with biopsy-verified skin vasculitis and 1 case each with temporal arteritis/polymyalgia rheumatica, necrotising panniculitis, Hashimoto's thyroiditis, autoimmune thrombocytopenia, and Sweet's syndrome. survival times, sex ratio and distribution of MDS subtypes were similar in the two groups. The patients in group A were younger than those in group B (median 66 vs. 76 years, p < 0.01). Four patients (40%) in group A had a history of previous genotoxic therapy for malignant disorders. The bone marrow karyotype was evaluated in 62 patients. Clonal chromosomal abnormalities were found more frequently in Group A than in group B (8/9 vs. 26/53, p = 0.03), and complex karyotypes, i.e., three or more aberrations, were also observed to be more common in group A (3/9 vs. 8/53). The results indicate that IMC preferentially develop in patients with secondary MDS, in younger MDS cases, and in patients with cytogenetic abnormalities.
- - - - - - - - - -
ranking = 0.00030752725908508
keywords = bone
(Clic here for more details about this article)

8/11. Parallel tubular granules in human immature neutrophils--an electron microscopic study.

    Unique rounded granules consisting of clustered parallel tubules, 29-31 nm in diameter, with occasional, flocculent or amorphous electron-dense material (parallel tubular granules, PTGs) were found in immature neutrophils in the bone marrow in a case of chronic neutrophilic leukemia (CNL). PTGs were positive for electron microscopic myeloperoxidase. Since no structures similar to PTGs have been documented, we investigated the bone marrow of 65 adult patients with hematologic diseases, and 7 adult patients with miscellaneous non-hematologic diseases, by electron microscopy. The results showed that no PTGs were found in any cases other than the original CNL case. These findings suggest that PTGs are rare granules representing certain primary granules. In addition, the presence of PTGs might be helpful in diagnosing CNL.
- - - - - - - - - -
ranking = 0.00061505451817016
keywords = bone
(Clic here for more details about this article)

9/11. macrophage activation syndrome and rheumatic disease in childhood: a report of four new cases.

    A macrophage activation syndrome (MAS) developed in four children with chronic rheumatic diseases. The presentation included fever, hepatic and splenic enlargement, profound depression of blood counts, lowering of ESR, elevation of SGOT/PT and hypofibrinogenemia. The most characteristic sign of MAS was the presence in the bone marrow aspirate of well differentiated macrophages showing active haemophagocytosis with haematopoietic elements in their cytoplasm. Activation of the macrophage was also illustrated by high levels of monokines in the serum of 2 patients. This immuno-hematological process of unknown etiology can be triggered by ubiquitous events such as infections and treatment with anti-inflammatory drugs. It is a potentially lethal complication which should be diagnosed rapidly, since administration of high-dose steroids with discontinuation of potentially toxic drugs can induce remission. Cyclosporin A was effective in two patients and may be of value in the management of the macrophage-activation syndrome. Its efficacy supports the central involvement of a T-cell dysfunction. It must be borne in mind that children with rheumatic diseases, especially the systemic form of juvenile chronic arthritis, are highly vulnerable to life-threatening macrophage activation, which appears to be more frequent than previously recognized. Very careful monitoring of apparently "innocent" drugs and intercurrent viral infections is thus required.
- - - - - - - - - -
ranking = 5.0003075272591
keywords = macrophage, bone
(Clic here for more details about this article)

10/11. immunoblastic lymphadenopathy: case report and literature review.

    A patient with immunoblastic lymphadenopathy (IL) had an unusual course of illness, with frequent episodes, over a 20-month period, of chills, fever, abdominal pain, hepatosplenomegaly and weight loss. The episodes were short-lived and many resolved spontaneously. Eventually generalized lymphadenopathy and profound monoclonal IgG gammopathy developed, with atypical mononuclear cells in the peripheral blood and increased numbers of plasmacytoid cells in the bone marrow. Lymph node biopsy revealed the morphologic triad typical of IL: proliferation immunoblasts, proliferation of small blood vessels and the deposit of an amorphous acidophillic material in the vascular walls and the interstitium. Up to October 1976 110 cases had been reported of this disorder, first described 3 years ago, which indicates that IL is not rare. Remissions have occurred spontaneously and after steroid therapy or chemotherapy or both. However, death has been reported in almost 50% of the cases, and the best approach to therapy remains to be determined.
- - - - - - - - - -
ranking = 0.00030752725908508
keywords = bone
(Clic here for more details about this article)
| Next ->


Leave a message about 'Immune System Diseases'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.