Sjogren’s syndrome (SS) is an autoimmune disorder and can cause many organic changes. Although disease pathogenesis has not yet been fully elucidated, substantial data has been demonstrated for diagnosis. Over the past 15 years, two sets of criteria have been published.
New 2016 ACR/EULAR classification criteria for primary Sjogren’s syndrome (SS) have been developed and endorsed by the ACR. Pulmonary involvement in SS include xerotrachea, bronchial sicca, obstructive small airway disease, interstitial lung diseases, lymphproliferative lung disease, pulmonary hypertension, pleurites, pleural effusion, and thickened pleura. Cardiac involvement includes pericarditis, pericardial effusion, and atrioventricular conduction block. Read More>>>>>>>>
The standard view of blood coagulation is based on the idea that a “cascade” of enzymatic events, the “intrinsic” and “extrinsic” pathways of coagulation both generating thrombin, which converts fibrinogen into an insoluble fibrin clot.
Consequently, much of the biomedical focus relating to blood coagulation has been on the clotting factor thrombin (clotting factor IIa), resulting from the “trypsinic” cleavage of prothrombin (clotting factor II) into thrombin (clotting factor IIa). For example, citrate is added to donated blood units to chelate Ca(II), which prevents the activation of carboxylated pro-factors (clotting factors II,VII, IX, X) into enzymatically functioning proteins (factors IIa, VIIa, IXa, Xa respectively). Read More>>>>>>>
Atypical Presentation of Myeloid Sarcoma Mimicking a Subcutaneous Hematoma as Complication of MDS and AML
About less than 1% of patients with CML or acute leukemic transformations of other chronic disorders such as myeloproliferative neoplasms, particularly myelofibrosis, will present with and extramedullary presentation. These presentations are known as myeloid sarcoma, also called granulocytic sarcoma, myeloblastoma, or chloroma.
When found in association with blood or bone marrow involvement, it occurs most commonly as either cutaneous or gingival infiltration by leukemic cells. However, sites of isolated myeloid sarcoma include bone, periosteum, soft tissues, and lymph nodes, and less commonly the orbit, intestine, mediastinum, epidural region, uterus, and ovary. Read More>>>>>>>>>>
WHO 2016 guidelines regarding chronic myeloid leukemia (CML) do not contain groundbreaking changes. Mainly criteria of acceleration phase (AP) identification were revised. Despite these changes, the guidelines are still not standardized and differ significantly, even when compared to guidelines of European Leukemia Net (ELN), International Bone Marrow Transplant Registry (IBMTR) or M. D. Anderson Cancer Center, to give some examples.
Compared to previous editions of the WHO classification, new parameters appeared, the presence of which requires identification of acceleration phase. In this case, one should list e.g. chronic leukocytosis (>10 × 109 /L), non-responding to treatment, chronic splenomegaly non-responding to treatment, additional clonal chromosomal aberrations (the so-called ” major route” ) in Ph+ cells on diagnosis. Read More>>>>>>>>>>
Thrombotic Thrombocytopenic Purpura Refractory to Plasmapheresis Treated Successfully with Vincristine
Thrombotic Thrombocytopenic Purpura (TTP) has a specific pathogenetic defect characterized by the inability to cleave ultralarge vWF (ULvWF). They are normally cleaved by a metalloprotease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 domain 13).
Low ADAMTS13 activity can result from a constitutive deficiency or circulating acquired IgG autoantibodies to different antigenic regions of the ADAMTS13 molecule in patients with acute TTP. The pathogenetic role of this autoantibody is supported by its disappearance from the circulation when remission is achieved by effective treatment. Read More>>>>>>>
Amyloid cardiomyopathy is a rare disease characterized by the extracellular deposition of insoluble fibrils resistant to proteases. It may be secondary to chronic inflammatory conditions, hereditary diseases or to the production of a light chain of monoclonal immunoglobulin.
This often leads to an infiltrative cardiomyopathy with a restrictive pathophysiology, conventionally associated with significant morbidity and poor prognosis: 30% survival at 2 years and a life expectancy of six months without treatment. Some cases had described the association between cardiac amyloidosis and multiple myeloma. However, the interest of this observation is related to the rarity of this association, the diagnosis difficulty of cardiac amyloidosis and more to find its etiology. Read More>>>>>>
Leukemia’s classification has been based traditionally on morphological features which are often subject to personality’s skills of the investigator. In the last years there were different methods for this such as cytochemical analysis, immunophenotyping and enzyme abnormalities have been used in the classification and subtyping of leukemic cells.
According to disturbances which occur in acute leukemia result from the cell destruction and over growth of up normal malignant cell. There was glycolytic enzymes lactate dehydrogenase (LDH), which it was distributed in all organs but, mainly found in liver and represent as necessary factor in gluconeogenesis process where it responsible for acceleration of convert pyruvate into lactate to which act as fuel for energy. Read More>>>>>>>>>>