IM - Hematology Oncology - Lymphomas and Plasma Cell Disorders

tonystep1's version from 2017-08-03 01:51


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Multiple Myeloma Skeletal manifestationsa. Bone pain due to osteolytic lesions, fractures, and vertebral collapse-occurs especially in the low back or chest (ribs) and jaw (mandible) b. Pathologic fractures c. Loss of height secondary to collapse of vertebrae
Multiple Myeloma Anemia(normocytic normochromic)-present in most patients due to bone marrow infiltration and renal failure
Multiple Myeloma Renal failurea. Myeloma nephrosis-Immunoglobulin precipitation in renal tubules leads to tubular casts of Bence Jones protein. b. Hypercalcemia also plays a role in renal decompensation
Multiple Myeloma Most common cause of deathRecurrent infections // Secondary to deprivation of normal immunoglobulins; therefore, humoral immunity is affected
Multiple Myeloma develops in 1 0% of patients (usually clinically insignificant)Amyloidosis
Waldenstriim's Macroglobulinemialymphadenopathy, splenomegaly, anemia, abnormal bleeding, and hyperviscosity syndrome (due to elevated IgM)
Monoclonal Gammopathy of Undetermined Significance IMGUS• Common in the elderly (up to 10% in patients >75 years of age) • Usually asymptomatic Fewer than 20% develop multiple myeloma in 10 to 15 years.


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Diagnostic criteria for multiple myelomaBone marrow biopsy reveals at least 1 0% abnormal plasma cells. plus one of the following: Serum monoclonal protein or urine monoclonal protein or Lytic bone lesions (well-defined radiolucencies on radiographs)predominantly found in the skull and axial skeleton
Waldenstriim's MacroglobulinemiaDiagnosis: IgM > 5 g/dL; Bence Jones proteinuria in 10% of cases; absence of bone lesions
Monoclonal Gammopathy of Undetermined Significance IMGUS)Diagnosis: IgG spike <3.5 g; less than 10% plasma cells in bone marrow; Bence Jones proteinuria < 1 g/24 hours


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Multiple Myelomal. Treatment is usually reserved for patients with symptoms or advanced disease. Indications for treatment include hypercalcemia, bone pain, and spinal cord compression. 2. Systemic chemotherapy-preferred initial treatment (alkylating agents) 3. Radiation therapy-if no response to chemotherapy and if disabling pain is present 4. Transplantation-Autologous peripheral blood stem cell transplantation is preferred over bone marrow transplantation
Waldenstriim's MacroglobulinemiaThere is no definitive cure. Use chemotherapy and plasmapheresis for hyperviscosity syndromes.
Monoclonal Gammopathy of Undetermined Significance IMGUS)No specific treatment is necessary, just close observation.Fewer than 20% develop multiple myeloma in 10 to 15 years.


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Hodgkin's Diseasel . Most common symptom is a painless lymphadenopathy // 2. Supraclavicular, cervical, axillary, mediastinal lymph nodes // 3. Spreads by continuity from one lymph node to adjacent lymph nodes // 4. Other presentations may or may not be present, including B symptoms (fever, night sweats, weight loss), pruritus, and cough (secondary to mediastinal lymph node involvement) .
Non·Hodgkin's Lymphoma (NHL)l. Lymphadenopathy-sometimes the only manifestation of disease // B symptoms-less common than in Hodgkin's lymphoma // Hepatosplenomegaly, abdominal pain, or fullness // Recurrent infections, symptoms of anemia or thrombocytopenia-due to bone marrow involvement // Various other findings are possible, e.g. , superior vena cava obstruction, respiratory involvement, bone pain.
Small Lymphoctic LeukemiaIndolent or Small lymphocytic • Closely related to CLL; • Eventually results in low-grade lymphoma more common in elderly patients widespread lymph node • Indolent course involvement with dissemination to liver, spleen, and bone marrow
Follicular, predominately small , cleaved cell lymphomaMost common form of NHL // Mean age of onset is 55 // May transform into diffuse large cell; associated with translocation t(14;18) // indolent course // presents with painless peripheral lymphadenopathy
Diffuse, large- cell lymphomaPredominately B-cell origin // Middle-aged and elderly patients // Locally invasive ; presents as large extranodal mass
Lymphoblastic lymphomaT cell lymphoma ; more common in children // May progress to T- ALL // 50% of patients have B symptoms
Burkitss's (small non-cleaved cell) lymphomaB-cell lymphoma more in children // African type involves facial bone and jaw // American type involves abdominal organs (hepatomegaly , abdominal masses, lymphadenopathy) // African variety linked with EBV infection // Associated with specific translocation t(8:14)
Mycosis fungoidesT cell lymphoma of the skin // Presents with eczematoid skin lesions that progress to generalized eythryoderma // Cribiform shape of lymphocytes // Disseminate to lymph nodes, blood and other organs
Sezary Syndromeinvolves skin as well as blood stream
HIV-associated lymphomasNot a discrete entity // usualy Burkitss or diffuse, large cell lymphoma// very poor prognosis


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Hodgkin's Diseasel. Lymph node biopsy-The presence of Reed Sternberg cells is required to make the diagnosis.// 2. Presence of inflammatory cell infiltrates-This distinguishes Hodgkin's lymphoma from non-Hodgkin's lymphoma (NHL). // CXR and CT scan (chest, abdomen)-to detect lymph node involvement // Bone marrow biopsy-to evaluate bone marrow involvement // Laboratory findings-leukocytosis, eosinophilia; level of ESR elevation sometimes corresponds with disease activity
Non·Hodgkin's Lymphoma (NHL)l. Lymph node biopsy-for definitive diagnosis. Any lymph node > l cm present for more than 4 weeks that cannot be attributed to infection should be biopsied.// a. CXR-may reveal hilar or mediastinal adenopathy // b. CT scan (chest, abdomen, pelvis) // c. Serum LDH and {32 microglobulin are indirect indicators of tumor burden.// d. If alkaline phosphatase is elevated, bone or liver involvement is likely. // e. If liver function tests or bilirubin is elevated, liver involvement is likely. // f. CBC // g. Serum electrolytes, renal function tests // h. Bone marrow biopsy
Hodgkin's Disease Stage Iconfined to single lymph node
Hodgkin's Disease Stage IIinvolvement of two or more lymph nodes but confined to same side of diaphragm
Hodgkin's Disease Stage IIIboth sides of diaphragm involved
Hodgkin's Disease Stage IVdissemination of disease to extralymphatic sites
Non-Hodgkin's Stage IHingle lymph node involved (or one extralymphatic site)
Non-Hodgkin's Stage IItwo or more lymph nodes on the same side of the diaphragm (or localized involvement of one lymph node region and a contiguous extralymphatic site)
Non-Hodgkin's Stage IIIlymph node involvement on both sides of the diaphragm
Non-Hodgkin's Stage IVdisseminated involvement of one or more extralymphatic organs with or without lymph node involvemen


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Hodgkin's Lymphomaconsists mainly of chemotherapy and radiation therapy to the involved field. l. Stages I, II, and IliA can be treated with radiotherapy alone. However, some physicians advocate the use of chemotherapy in these patients as well. 2. Stages IIIB and IV require chemotherapy.
Non·Hodgkin's Lymphoma (NHL) Indolent formsIndolent forms of NHL are not curable, but have a 5-year survival rate of 75%.// These patients are treated in a variety of ways, depending on the patient's age, comorbidities, stage of disease, and wishes, as follows.a. Observation b. Chemotherapy (single-agent or combination) c. Radiation therapy
Non·Hodgkin's Lymphoma (NHL) intermediate and high grade forms Intermediate and high-grade NHLs may be curable with aggressive treatments, but if complete remission is not achieved, survival is usually less than 2 years. In general, aggresive forms are treated with multiple regimens of combination chemotherapy (e.g. CHOP) and radiation therapy.
What s CHOP therapyCyclophosphamide // Hydroxydaunomycin (doxorubicin) // Oncovin (vincristine) // Prednisone