Blood L6: Plasma Cell Diseases PDF

Data Zone 40 Blood , Medicine ||| Blood L6 : Plasma Cell diseases Under normal circumstances, maturation of B lymphoctes to antibody secreting plasma cells is stimulated by exposure to the antigen for which the surf...

Data Zone 40 Blood , Medicine ||| Blood L6 : Plasma Cell diseases Under normal circumstances, maturation of B lymphoctes to antibody secreting plasma cells is stimulated by exposure to the antigen for which the surface immunoglobulin is specific. monoclonal neoplasms related to each other by the fact that they are developed from common progenitors in the B lymphocyte lineage, The clinical manifestations of all the plasma cell disorders relate to: a) expansion of the neoplastic cells b) secretion of cell products (immunoglobulin molecules or subunits, lymphokines) c) host's response to the tumor. Classification: I. Monoclonal gammopathy of undetermined significance (MGUS) II. Multiple myeloma. III. Plasma cytoma. IV. Immunoglobulin deposition diseases: a) Primary amyloidosis. AL protein b) Systemic light and heavy chain deposition diseases. V. Osteosclerotic myeloma (POEMS) : The features of this syndrome are polyneuropathy, organomegaly, endocrinopathy, multiple myeloma, and skin changes. ALP high in metastatic carcinoma But normal in MM 1 Data Zone 40 Blood , Medicine ||| Multiple myeloma  Def : malignant proliferation of plasma cells derived from a single clone.  Common above age of 60y.  CP Hypercalcemia Hypercalcemia Bone pain is the most common symptom in myeloma, affecting nearly 70% of patients. Renal affection The pain usually involves back and ribs, and unlike pain of metastatic carcinoma, which often is worse at night, the pain of myeloma is precipitated Anemia by movement. osteoporosis, pathologic fractures, lytic bone lesions also occur. Punched out Renal affection Renal failure occurs in nearly 25% of myeloma patients and abnormal renal pathology is noted in over half Pathogenesis : Hypercalcemia, light chain deposition, amyloidosis, urate nephropathy, drug toxicity (NSAID, bisphosphonates), contrast dye Renal dysfunction due to light chain deposition disease (light chain cast nephropathy) & amyloidosis. Anemia Anemia occurs in 80% of myeloma patients It is usually normocytic and normochromic. Pathogenesis: Bone marrow infiltration, production of inhibitory factors, hemolysis, red cell production, decreased erythropoietin levels 2 Data Zone 40 Blood , Medicine ||| Others : Recurrent infection. the second common presentation in patient é myeloma is susceptibility to bacterial infections. The most common infections are pneumonias and pyelonephritis, the most frequent pathogens are Streptococcus pneumoniae, Staphylococcus aureus and Klebsiella pneumoniae in the lungs and Escherichia coli and other gram-negative organisms in the urinary tract. Neurologic symptoms: - Un common - Causes: Hyperviscosity, cryoglobulinemia, amyloid deposits, hypercalcemia, nerve compression, anti-neuronal antibody, POEMS syndrome, therapy-related toxicity. Hepatosplenomegaly and lymphadenopathy are rare. Hyperviscosity syndrome ; occurs in 2% of cases. Investigation : I) CBC: 1) Normocytic normochromic anemia. 2) Rare patients II) Erythrocyte sedimentation rate(ESR) is elevated. III) Blood chemistry: 1) Raised serum calcium (40%) , urea nitrogen, creatinine, and uric acid levels. 2) Serum alkaline phosphatase is usually normal even with extensive bone involvement because of absence of osteoblastic activity. 3) Quantification of serum B2-microglobul in Levels below 3.5mg/L imply good prognosis and above 5.5mg/L imply bad prognosis. 3 Data Zone 40 Blood , Medicine ||| IV) Protein electrophoresis : Protein electrophoresis and measurement of serum immunoglobulins and free light chains are useful for detecting and characterizing M spikes. V) 24-h urine protein excretion é electrophoresis and immunologic typing of any M component: The urine contains free light chains, Bence-Jones protein in 2/3 of patients. VI) Marrow plasmacytosis (> 10%). Bone marrow plasma cells are CD138+ and monoclonal. VII) Radiological Investigations: Chest and bone radiographs may reveal lytic lesions or diffuse osteopenia. The classic triad of myeloma : a)Marrow plasmacytosis (> 10%). LYTIC BONE LESION SERUM AND /OR URINE M COMPONENT 4 Data Zone 40 Blood , Medicine ||| TREATMENT: SUPPORTIVE TREATMENT : 1) Hypercalcemia: - Bisphosphonates, glucocorticoid therapy, hydration, and natriuresis. - Calcitonin may add to inhibitory effects of glucocotticoids on bone resorption. 2) Renal disorders : - maintaining a high fluid intake to prevent dehydration and to help excrete light chains and calcium. - Treatment of acute renal failure - Plasmapheresis is very effective in clearing light chains. - Reduction of protein load by effective antitumor therapy é agents such as brotezomib may result in functional renal improvement. 3) Plasmapheresis : the treatment of choice for hyperviscosity. 4) Control of pain. 5) Treament of anemia. SPECIFIC TREATMENT : 1) Allogenic stem cell transplantation (patients under 50 years with no comorbid diseases) This is preceded by intensive chemotherapy. 2) Autologous stem cell transplantation : After several courses of chemotherapy, when number of tumor cells has been markedly reduced. 3) Chemotherapy. ‫ اللهم فرج‬،‫ اللهم ارحم إخواننا المسلمين المستضعفين في كل مكان‬- ‫همهم ونفس كربهم وأقل عثراتهم وتول بنفسك أمرهم وارفع رايتهم‬.ً‫واكبت عدوهم ووحد صفهم واجمع كلمتهم وردهم إليك رداً جميلا‬ ‫ واجعل‬،‫ اللهم من أراد الإسلام والمسلمين بسو ٍء فأشغله في نفسه‬-.‫تدبيره تدميراً عليه‬.‫ ونعوذ بك من شرورهم‬،‫ اللهم عليك بأعدائنا فإنهم لا يعجزونك‬- 5