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Treatment with sirolimus results in complete responses in patients with autoimmune lymphoproliferative syndrome. Summary We hypothesized that sirolimus, an mTOR inhibitor, may be effective in adaah with autoimmune lymphoproliferative syndrome ALPS and treated patients who were intolerant to or failed other therapies.

Four patients were treated for autoimmune cytopenias; all had a rapid complete or near complete response. Two patients were treated for autoimmune arthritis and colitis, demonstrating marked improvement. Three patients had complete resolution of lymphadenopathy and splenomegaly and all patients had a reduction in double negative T cells, a population hallmark of the disease.

Based on these significant responses, we recommend that sirolimus be considered as second-line therapy for eritropoiessis with steroid-refractory disease. Directory of Open Access Journals Sweden.

Full Text Available Multicentric Castleman’s disease MCD is lymphoproliferative disorder characterized by systemic inflammatory symptoms eritropoieeis as fever and weight loss. IgG4-related disease in autoimmune lymphoproliferative syndrome.

In retrospect, he had high levels of serum IgG4 for several years prior to presenting with IgG4-related pancreatitis. Hence, IgG4-related disease should be considered in ALPS patients, especially in those manifesting lymphocytic organ infiltration or excessive hypergammaglobulinaemia. As a result, the normal homeostasis of T- adalxh B-lymphocytes is disturbed and a proliferation of polyclonal T lymphocytes occurs.

Somatic FAS mutations are common in patients with genetically undefined autoimmune lymphoproliferative syndrome. Autoimmune lymphoproliferative syndrome ALPS is characterized by childhood onset of lymphadenopathy, hepatosplenomegaly, autoimmune cytopenias, elevated numbers of double-negative T DNT cells, and increased risk of lymphoma.

We sought to determine the proportion of patients with somatic FAS mutations among a group of our ALPS patients with no detectable germline mutation and to further characterize them. We found more than one-third 12 adalha 31 of the xdalah tested had somatic FAS mutations, primarily involving the intracellular domain of FAS resulting in loss of normal FAS signaling. Post-transplant lymphoproliferative disorder in adlaah pelvis successfully treated with consolidative radiotherapy.

Post-transplant lymphoproliferative disorders PTLDs are aggressive malignancies which represent one of the major post-transplant complications. However, treatment options vary significantly and localized disease may be curatively treated with radiotherapy RT or surgery. We describe a patient who developed a rectal lymphoproliferative lesion 11 years after kidney transplant, which was successfully treated with consolidative RT using RT was well tolerated and the patient showed no signs of grade 3 or 4 toxicity.

This patient is free of recurrence 52 months after RT, with an overall survival of 62 months since diagnosis. Conventionally fractionated adalay RT appears to be a tolerable and effective treatment option for localized PTLD if a sufficient systemic treatment cannot be applied. Die RT wurde komplikationslos vertragen und es zeigten sich keine Nebenwirkungen. Das rezidivfreie Ueberleben betrug zum Zeitpunkt der letzten Nachsorgeuntersuchung 52 Monate mit einer Gesamtueberlebenszeit von 62 Monaten seit der Diagnose.

Die konventionelle fraktionierte moderat dosierte RT scheint eine gut. Development of an Epstein-Barr virus-associated lymphoproliferative disorder in a patient eritropoiexis with azacitidine for chronic myelomonocytic leukaemia.

Some chemotherapeutic agents can cause iatrogenic lymphoproliferative disorders. In analogy eritropoiesiss what has been observed with other nucleoside analogues such as cladribine and fludarabine, we document the first case of an Epstein-Barr virus-positive, iatrogenic immunodeficiency-associated, lymphoproliferative disease, formally resembling polymorphic post-transplant lymphoproliferative disease in a patient treated with azacitidine Vidaza for chronic myelomonocytic leukaemia CMML.

CDK Edisi 270 – Neurologi

A year-old female adalan was diagnosed with CMML in Januaryand treatment with azacitidine was initiated, which lasted for five cycles from February until June The patient was hospitalized in June under the suspicion of pneumonia. Transformation of the CMML was suspected at that time too. During hospitalization, a generalized enlargement of the lymph nodes and the spleen was noticed.

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The patient rapidly deteriorated and finally died of respiratory insufficiency.

GINJAL – Definition and synonyms of ginjal in the Malay dictionary

At autopsy, an Epstein-Barr virus-associated lymphoproliferative disorder, resembling polymorphic post-transplant lymphoproliferative disease with involvement of the lymph nodes, the spleen and the lung and causing necrotizing pneumonia, was afalah. Diagnostic criteria for diffuse large B-cell lymphoma or infectious mononucleosis-like lymphoproliferative disease were not met.

This is the first documented case of an azacitidine-associated lymphoproliferative disease, raising awareness for possible not yet known side effects of this drug, which should be kept in mind by oncologists and pathologists. A year-old Chinese boy case report. Autoimmune lymphoproliferative syndrome ALPS usually presents in childhood with fever, nonmalignant splenomegaly and lymphadenopathy along with hemocytopenia. This case report describes a year-old boy presenting with signs of autoimmune disease, splenomegaly, hepatomegaly and resistant hemocytopenia.

Sirolimus controlled the relapsed thrombocytopenia after splenectomy. Sequencing of the FAS gene identified two spontaneous heterozygous mutations c. The boy’s homozygous missense variation c. Published by Elsevier B. Frequency of a FAS ligand eritropoiseis variant associated with inherited feline autoimmune lymphoproliferative syndrome in British shorthair cats in New Zealand.

Pedigree analysis was performed and inbreeding coefficients were calculated for cats with the FASLG variant. For an inherited disease, lethal at a young age, in eritroppiesis small population in which inbreeding is common, these results are significant.

Treating autoimmune disorders with venom-derived peptides. The effective treatment of autoimmune diseases remains a challenge. Preclinical and clinical studies have produced a wealth of information on Kv1.

This review adalzh the advances in screening and design of these peptides with erotropoiesis structures and potencies. It focuses on representative strategies for improving peptide selectivity and discusses the preclinical research on those venom-derived peptides as well as their clinical developmental status.

Encouraging results indicate that peptides isolated from the venom of venomous animals are a large resource for discovering immunomodulators that act on Kv1. Since the structural diversity of venom-derived peptides determines the variety of their pharmacological activities, the design and optimization of venom-peptides for improved Kv1.

These advances should further accelerate research, development eritroppoiesis the future clinical application of venom-derived peptides erritropoiesis targeting Kv1.

Pulmonary aspergillosis and central nervous system hemorrhage as complications of autoimmune hemolytic anemia treated with corticosteroids. Warm, active antibody adult autoimmune hemolytic anemia is the most common form of hemolytic anemia not related to drug therapy. Mortality in adult autoimmune hemolytic anemia is related to the inability to successfully treat patients’ underlying disease, or the infectious complications of splenectomy and prolonged steroid therapy.

Predisposing factors for invasive aspergillosis are neutropenia and steroid therapy. We present a fatal case of aspergillosis complicating a nonneutropenic case of warm active antibody adult autoimmune hemolytic anemia treated with prolonged steroid therapy. Autoimmune lymphoproliferative syndrome and non-Hodgkin lymphoma: Suggestions from a case report.

A young patient with undefined autoimmune lymphoproliferative syndrome ALPS-U and low back pain underwent a CT and MRI study that showed enhancing vertebral lesions, some pulmonary nodules and diffuse latero-cervical lymphadenopathy. A bone marrow biopsy showed a “lymphomatoid granulomatosis”, a rare variant of B-cell non-Hodgkin lymphoma NHL. Full Text Available Early-stage apoptotic cells possess immunomodulatory properties.

Proper apoptotic cell clearance during homeostasis has been shown to limit eritropoieis immune responses. Based on these observations, early-stage apoptotic cell infusion has been used to prevent unwanted inflammatory responses in different experimental models of autoimmune diseases or transplantation. Moreover, this approach has eritropoidsis shown to be feasible without any toxicity in patients undergoing allogeneic hematopoietic cell transplantation to prevent graft-versus-host disease.

However, whether early-stage apoptotic cell infusion can be used to treat ongoing inflammatory disorders has not been reported extensively. Recently, we have provided evidence that early-stage apoptotic cell infusion is able to control, at least transiently, ongoing collagen-induced arthritis. Furthermore, the efficacy of this approach is not efitropoiesis by the association with two standard treatments of rheumatoid arthritis RA, methotrexate and tumor necrosis factor TNF inhibition.


Here, in the light of these observations and recent data of the literature, we discuss the mechanisms of early-stage apoptotic cell infusion and how this therapeutic approach can be transposed to patients with RA. Eritropoisis management of steroid-induced hyperglycaemia and steroid-induced diabetes in people with lymphoproliferative disorders treated with intermittent high dose steroids.

Eighty-three people were diagnosed with a lymphoproliferative disorder, of whom 6 had known Type 2 diabetes. Fifty-three people without known diabetes were screened by HbA1c and random venous plasma glucose.

All 7 people writropoiesis Type 2 diabetes developed SIH and 3 required insulin. Autoimmune diseases are characterized by overactive immunity, where the body’s defense system launches an eritdopoiesis against itself. If left unchecked, this can result in the destruction erirropoiesis healthy tissue and significantly affect patient well-being.

In the case of type II autoimmune hypersensitivities, autoreactive antibodies attack the host’s own cells or extracellular matrix. Current clinical treatment modalities for managing this class of disease are generally nonspecific and face considerable limitations.

In this Topical Review, we cover emerging therapeutic strategies, with an emphasis on novel nanomedicine platforms. Specifically, the use of biomimetic cell membrane-coated nanosponges that are capable of specifically binding and neutralizing pathological antibodies will be explored. There is significant untapped potential in the application of nanotechnology for the treatment of autoimmune diseases, and continued development along this line may help to eventually change the clinical landscape.

Free radical theory of autoimmunity. Full Text Available Abstract Background Despite great advances in clinical oncology, the molecular mechanisms underlying the failure of chemotherapeutic intervention in treating lymphoproliferative and related disorders are not well understood. Hypothesis A hypothetical scheme to explain the damage induced by chemotherapy and associated chronic oxidative stress is proposed on the basis of published literature, experimental data and anecdotal observations.

Brief accounts of multidrug resistance, lymphoid malignancy, the cellular and molecular basis of autoimmunity and chronic oxidative stress are assembled to form a basis for the hypothesis and to indicate the likelihood that it is valid in vivo.

Conclusion The argument set forward in this article suggests a possible mechanism for the development of autoimmunity. According to this view, the various sorts of damage induced by chemotherapy have a role in the pattern of drug resistance, which is associated with the initiation of autoimmunity.

No serious adverse event was noticed in both groups. A significant post-vaccine decrease in autoantibody positivity was observed. Moreover, HLA-A2 subjects have been analyzed for the possible CD8T-cell response to apoptotic self-epitopes, without observing significant difference between baseline and one month post-vaccine. Full Text Available Chemokines and their receptors play critical roles in the progression of autoimmunity and inflammation.

Typically, multiple chemokines are involved in the development of these pathologies. Indeed, targeting single chemokines or chemokine receptors has failed to achieve significant clinical benefits in treating autoimmunity and inflammation. Moreover, the binding of host atypical chemokine receptors to multiple chemokines as well as the binding of chemokine-binding proteins secreted by various pathogens can serve as a strategy for controlling inflammation.

These peptides were cloned into human mutated immunoglobulin Fc-protein fusions peptibodies. Furthermore, BKTFc and BKTFc also showed a significant inhibition of disease progression in a variety of animal models for autoimmunity and inflammation. Developing a novel class of antagonists that can control the courses of diseases by selectively blocking multiple chemokines could be a novel way of generating effective therapeutics.