Pathology 1 - Block 3 - Part 4

davidwurbel7's version from 2016-04-03 23:41

WBC & Lymph Nodes Part 6

Question Answer
Monoclonal immunoglobulin identified in bloodM Component
Neoplastic plasma cells synthesize excess of ‘L’ (light) chains. free L chains are known asBence-Jones Proteins
Bence-Jones proteins can be found hereUrine
Bence-Jones proteins present in the urine can be indication of this diseaseMultiple Myeloma
Identical molecule of Ig would migrate in serum protein electrophoresis to produceM Spike
This molecule migrated into the serum produces an M spike in serum protein electrophoresisImmunoglobulin (Ig)
Multiple myeloma, Waldenström macroglobinemia, Primary immune associated amyloidosis and Monoclonal gammapathy of undetermined significance (MGUS) are examples ofPlasma Cell Neoplasms
Waldenström macroglobinemia is also known asLymphoplasmacytic Lymphoma
Age dependent monoclonal tumor of bone marrow plasma cells often with significant end organ damage that can include lytic bone lesions, anemia, loss of kidney function, low immunity, and amyloid deposits. Incidence - average age at diagnosis: 71 years. higher risk among African AmericansMultiple Myeloma
This cytokine produced by neoplastic plasma cells and normal stromal cells in bone marrow is responsible for proliferation and survival of myeloma cells. If it is present in high levels, associated with poor prognosisIL-6
Neoplastic plasma cells produce MIP 1α which upregulates NF-kB ligand RANKL these serve asOsteoclast Activating Factors
Bone marrow involvement produces destruction of both medullary and cortical bone leading to pathological fractures, and hypercalcemiaOsteoclast Activating Factors
Risk Factors include occupational exposure such as radium, benzene, radiation. Earlier days there was an association with Thorostat. Others risk factors include cigarette smoking and hair dye (possibly)Multiple Myeloma
Complete blood counts - in the course of the disease, anemia, thrombocytopenia may occur due to marrow infiltration. Peripheral blood smear: Rouleaux formation (stack of coins) of red cells, increased viscosity due to presence of immunoglobulins with a pale blue background. Bone marrow: more than 30% plasma cells this is considered as a major criteria. marrow shows abnormal plasma cells, plasmablasts, and multinucleated plasma cellsMultiple Myeloma
Red cells sticking together due to increase Ig in the serum. The red cells appears like stack of coinsRouleaux formation
Lab diagnosis: Bleeding manifestations abnormal platelet function leads to prolonged bleeding time, uremia, increased hyperviscosity, interference with coagulation factors; ESR (Erythrocyte Sedimentation Rate): increased. Urine: proteinuria seen in 50 to 60% of cases, presence of light chains (L), Bence-Jones proteins, demonstrated by immune electrophoresis. Radioimaging: punched out lesions (osteolytic) spine, ribs, skull, pelvis, femur. Immunophenotype: abnormal plasma cells which show positivity for CD138, and CD56. Serum electrophoresis > 3gm/dL of Ig in serum and or > 6gm/dL of Bence-Jones protein in urineMultiple Myeloma
Renal failure seen in 50% of the patients deposition of proteinaceous tubular casts (Bence-Jones protein). Nephrocalcinosis: metastatic calcification. Amyloidosis: light chains are converted to amyloid (AL type)Multiple Myeloma
Renal failure seen in multiple myeloma is due to thisAmyloid Deposition
Asymptomatic, detected incidentally. isolated M spike (less than 3 gm/dL), no lytic bone lesions, no hypercalcemia, no Bence Jones protein. Bone marrow shows less than 10% plasma cellsMonoclonal Gammopathy of Uncertain Significance (MGUS)
Disease seen in older population, 6th to 7th decade of life. Malignancy of B-cells characterized by monoclonal IgM (complete)Lymphoplasmacytic Lymphoma
The Pathology of this disease acquired mutation in MYD88. Malignant lymphoplasmactyoid cells secrete monoclonal IgM. Major manifestation: hyperviscosity due to increase monoclonal IgM (pentamer)Lymphoplasmacytic Lymphoma
Heavy and light chain synthesis is balanced. No complications arising from secretion of light chains. Tumor dissemination to lymph nodes, spleen and liver. Infiltration of nerve roots and meninges may be seen later in the course of the disease. Serum calcium levels: normalLymphoplasmacytic Lymphoma
Lab diagnosis CBC: normal; Peripheral blood smear: rouleaux seen; Bone marrow: increase in lymphoid cells. Intermediate forms between lymphocytes and plasma cells noted (lymphoplasmacytic cells). Plasma cells are also present. Russell bodies may be seen. Increase in mast cells (reactive) Lymphoplasmacytic Lymphoma
Clinical features include weakness, weight loss, visual disturbances, hemostatic disturbance. Retinal: hemorrhages, cotton wool exudates. Lymphadenopathy, hepatomegaly, splenomegaly, hyperviscosity, anemia due to marrow infiltration. Autoimmune hemolytic anemia in 10%: presence of ‘cold agglutinins’ due to IgM. Raynaud’s phenomenon may be seen peripheral neuropathy: monoclonal IgM have high affinity for myelin. May be preceded by MGUS. Incurable progressive diseaseLymphoplasmacytic Lymphoma
Langerhans cell areImmature Dendritric Cells
Contain racquet shaped structures: Birbeck granules. S-100 positive, CD1a positive. Normally present in skin, oral mucosa. Derived from bone marrowLangerhans Cell Histiocytosis
Proliferation of immunophenotypically and functionally immature, rounded Langerhans cells. Along with eosinophils, macrophages, lymphocytes, and, sometimes, multinucleated giant cells. Electron microscopy demonstrates Birbeck granules, which are rod- or racquet-shaped cytoplasmic structures, seen in 50%Langerhans Cell Histiocytosis
Clinical features include before 2 years of age, cutaneous lesions trunk, scalp, hepatosplenomegaly, lymphadenopathy, lung, osteolytic lesions: skull, pelvis, long bones. Anemia/thrombocytopenia. Repeated infections. fatalMultifocal Multisystem
Clinical features include seen in children. Presents with fever, localized rash on scalp and in ear canal. triad: lytic lesions in skull, diabetes insipidus (invasion of posterior pituitary stalk), and exophthalmos (infiltration of the orbit)Hand-Schuller-Christian
Clinical features include benign histiocytosis. Seen in adolescents and adults. unifocal lytic lesions skull, ribs, femur. Excellent prognosisEosinophilic Granuloma

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