Plasma cell dyscrasias

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Multiple myeloma and solitary plasmacytoma - a radiological assessment compiled from Yochum

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Plasma cell dyscrasias:

Dr. Varun Babu Plasma cell dyscrasias

Multiple Myeloma:

Multiple Myeloma Dr. Varun Malignant proliferation of plasma cells – Kahler’s disease Young physician and chemist Henry Bence Jones described the protein in detail MC primary malignant tumor of bone. 50-70 yrs of age. M:F = 2:1

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Dr. Varun Lateral Skull. Note the multiple discrete osteolytic lesions distributed throughout the calvaria (raindrop skull)

SIGNS AND SYMPTOMS:

SIGNS AND SYMPTOMS Dr. Varun Anemia: replacement or alteration of hematopoietic tissues by proliferating plasma cells Deossification of bones housing red marrow Production of abnormal serum and urinary proteins Renal disease Pain is usually the presenting symptom weight loss, cachexia , anemia, unexplained osteoporosis

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A. Lateral Lumbar Spine. Observe the gross osteoporosis throughout the lumbar spine. There is a compression fracture of the superior endplate of L2 ( arrow ). The radiopaque densities in the spinal canal represent contrast material from a previous myelogram . B. Lateral Lumbar Spine. Note the diffuse osteoporosis throughout the entire lumbar spine. There are pathologic fractures of the T12 and L2 vertebral bodies ( arrows ). Dr. Varun

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Dr. Varun

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AP Thoracic. Observe the collapse of the T7 vertebral body ( arrow ). The pedicles have not been destroyed and are visualized on this frontal radiograph ( arrowheads ). B. Lateral Thoracic. Note the uniform collapse of the T7 vertebral body. Observe the collapse of the posterior third of the vertebral body, which strongly suggests a pathologic fracture, as was the case in this myeloma patient. This is a characteristic appearance for a vertebra plana or a wrinkled vertebra Dr. Varun

LOCATION:

LOCATION Dr. Varun Lower thoracic and lumbar spine Pelvis, skull, ribs, clavicle, scapula Diaphyses of femur and humerus .

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Dr. Varun

LAB:

LAB Dr. Varun Normocytic normochromic anemia, rouleaux formation, thrombocytopenia, elevated ESR Peripheral blood smear plasma cells – rare, except in plasma cell leukemia Hypercalcemia – lytic destruction of myelomatoid tissue. Normal serum phosphorus if not a/w renal disease Elevated plasma proteins. Hyperglobulinemia with A:G reversal (A:G reversal D/Ds – sarcoidosis , chronic nephritis, chronic cirrhosis, lymphogranuloma venereum )

LAB:

LAB Dr. Varun Serum electrophoresis: definitive examination in diagnosis 50% IgG ; 25% IgA ; 1-2% IgD . IgE , IgM are rare M spike Urine – Bence Jones protein Light immunoglobulin thermal behavior is peculiar that it first coagulates and then dissolves at temp >60deg. D/D: also seen in lymphoma, polycythemia vera , metastasis Hyperuricemia – accelerated nucleic acid metabolism Marrow: aspiration biopsy of sternum/iliac bone . >10% plasma cells Morula’s , Mott’s, flaming, thesaurocytes

PATHOLOGIC FEATURES:

PATHOLOGIC FEATURES Dr. Varun Osseous involvement Multiple, permeative lesions, filled by gelatinous, red soft masses of neoplastic plasma cells. These collections of plasma cells are surrounded by osteoclastic activity Round cell disorder – round to oval nuclei (D/D: NHL, Ewing’s) Kidney Protein precipitation in tubules – numerous hyaline casts Tubular damage 2 nd MC cause of death after pneumonia

PATHOLOGIC FEATURES:

PATHOLOGIC FEATURES Dr. Varun Amyloidosis 15% myeloma cases Circumscribed mass of amyloid around a joint – pad sign of secondary amyloid arthropathy Extraosseous plasmacytoma Nasopharynx , nasal cavity, oral cavity, tonsils, sinuses and larynx

RADIOLOGIC FEATURES :

RADIOLOGIC FEATURES Dr. Varun Radionuclide bone scans Cold scan except at sites of pathologic fracture. Release of osteoclast activating factor allows osteoclastic activity to predominate. Radionuclide uptake depends on osteoblastic activity

CONVENTIONAL RADIOGRAPHY:

CONVENTIONAL RADIOGRAPHY Dr. Varun Osteoporosis Generalized and severe osteoporosis most evident in lower dorsal and lumbar spine Diffuse loss of bone density with thinning of cortex. Vertebral body density similar to IVD Osteolytic defects Sharply circumscribed osteolytic defect. Punched out multiple round lesions Widespread lytic lesions of skull – raindrop skull (D/D: mets , non uniform lytic lesions)

CONVENTIONAL RADIOGRAPHY:

CONVENTIONAL RADIOGRAPHY Dr. Varun Spine Lower thoracic and lumbar spine Early osteoporosis, pathological vertebral collapse. Vertebra plana . May be single or multiple – wrinkled vertebrae of myeloma Pedicle sign – preservation of pedicles due to paucity of red marrow Pelvis and long bones Diffuse osteolytic round or oval lesions without reactive sclerosis Medullary bone destruction abuts the endosteal cortex Humerus and femur Symmetric diffuse lytic lesions

CONVENTIONAL RADIOGRAPHY:

CONVENTIONAL RADIOGRAPHY Dr. Varun Osteoblastic lesions <3% patients May be solitary or multiple Solitary ivory vertebra (D/Ds: osteoblastic metastases, mastocytosis , lymphoma, myelosclerosis )

DIAGNOSTIC CRITERIA:

DIAGNOSTIC CRITERIA Dr. Varun 10% of atypical, abnormal, or immature plasma cells in the bone marrow plus one of the following Serum M-protein spike Urine M-protein spike or Bence Jones proteinuria Characteristic osteolytic bone lesions Generalized osteoporosis Biopsy proven plasmacytoma

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Dr. Varun CT Define lesions and assess soft tissue components MRI Marrow infiltration. T1 dark. T2 heterogeneous increase in signal

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A. Initial Lesion. Note that a well-demarcated lesion with endosteal scalloping is readily appreciated ( arrow ). B. Post-radiotherapy. Observe that the lesion has become homogeneously sclerotic, characteristic of postradiation response. Dr. Varun

TREATMENT, PROGNOSIS :

TREATMENT, PROGNOSIS Dr. Varun Plasma cells – radiosensitive, converts to a blastic area Chemotherapy – Melphalan and Cytoxan ; prednisone Zoledronic acid to treat hypercalcemia

SOLITARY PLASMACYTOMA:

SOLITARY PLASMACYTOMA Dr. Varun Localized plasma cell proliferation 50% present before 50yrs. Mandible, ileum, vertebrae, ribs, proximal femur, scapula Typical: geographic radiolucent lesion, highly expansile , soap bubble internal architecture (D/D: pseudotumor of hemophilia, hydatid bone, fibrous dysplasia, GCT, brown tumor, blow out mets from kidney, thyroid. May progress to multiple myeloma

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Solitary plasmacytomas Dr. Varun

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