Page 1120 - Clinical Immunology_ Principles and Practice ( PDFDrive )

1084 Part eight Immunology of Neoplasia

KeY CONCePtS Management
Multiple Myeloma Patients who have end-organ damage attributable to the underly-

• A cancer of plasma cells. ing plasma cell disorder have MM and require therapy. The
• Organ damage due to plasma cell proliferation and M-protein following findings, if attributable to the underlying plasma
overproduction. cell disorder, are clear indications for treatment: anemia,
• Pathophysiology of multiple myeloma consists mainly of CRAB: hypercalcemia, renal insufficiency, lytic bone lesions or severe
hyperCalcemia, Renal, Anemia, and Bone. osteopenia, and extramedullary plasmacytoma (excluding
• History and physical examination, laboratory tests, bone marrow testing, SEP). Asymptomatic patients who have one of the follow-
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and imaging all play key roles in the diagnosis and management of
myeloma. ing biomarkers are also considered to have MM that requires
• Prognosis and treatment vary depending on risk stratification. therapy: ≥60% clonal plasma cells in the bone marrow, involved/
uninvolved FRL ratio of ≥100, more than one focal bone lesion
on MRI. 9
High-dose chemotherapy with autologous hematopoietic
stem cell transplantation (HSCT) is the preferred therapy
for patients with standard- or intermediate-risk myeloma.
Patients eligible for HSCT receive induction therapy for 2–4
months prior to stem cell collection to reduce the number
of tumor cells in the bone marrow and peripheral blood, to
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lessen symptoms, and to mitigate end-organ damage. The
preferred induction chemotherapy regimen depends on the risk
stratification.
Patients ineligible for HSCT receive induction lenalidomide
plus dexamethasone. In contrast, those receiving alkylator- or
bortezomib-based regimen are treated for approximately 12 to
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18 months and then observed until progression. Patients with
high-risk MM do poorly with all conventional treatment options.
All patients should be evaluated before each treatment cycle to
determine the degree of disease response.
Patients with one or more lesions on skeletal radiographs
and those with osteopenia should be given bisphosphonate
therapy. Pneumococcal and influenza vaccines should also be
given to all patients. Patients with MM frequently develop
complications related to their disease, including hypercalcemia,
renal insufficiency, infection, and skeletal lesions, which require
Fig 80.3 Photomicrograph of Plasma Cells in a Bone Marrow specific treatment. 9
Biopsy From a Myeloma Patient. Plasma cells show atypical Almost all patients with MM who survive initial treatment
features, including visible nucleoli, multinucleation, high nuclear/ will eventually relapse and require further therapy. Treatment
cytoplasmic ratios, and increased size. (Courtesy Dr. Mikhail options for patients with relapsed or refractory MM include
Roshal, Department of Pathology, Memorial Sloan Kettering HSCT, a reinitiation of the previous chemotherapy regimen, or
Cancer Center.) a trial of a new regimen. 9

A B
Fig 80.4 Conventional Radiographs Showing Myeloma Osteolytic Lesions in the (A) Skull
and (B) Humerus. (Courtesy Dr. Jonathan Landa, Department of Radiology, Memorial Sloan
Kettering Cancer Center.)