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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3063_Библиотеки_им_академика_М_И_Перельмана
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Final Diagnosis
Chronic lymphocytic leukemia
Fludarabine or bendamustine
Add rituximab for those CD20-positive (most)
Cyclophosphamide (often used with fludarabine and rituximab)
Chlorambucil in older, more fragile persons
371

Case 3
Chief Complaint
“My shoulder hurts.”
History and Physical Examination
A 75-year-old woman comes to the emergency department with severe pain in her left
shoulder. She states that she was leaning against a wall with her left arm when suddenly
she felt “something snap” in that arm. She states that over the last several months, she has
experienced intermittent back and rib pain but didn’t seek medical attention. She denies
fever and chills. There is no history of weight loss.
Her T is 37.0 C (98.6 F), BP 110/68 mm Hg, and pulse 76/min. She is unable to lift her
left arm. Physical examination is otherwise unremarkable.
Differential Diagnosis
CCS NOTE
Traumatic fracture
1.
Pathologic fracture (e.g., multiple myeloma, osteoporosis)
2.
Metastatic cancer (breast, lung, ovary)
3.
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In the ICU, examine only the areas surrounding the chief complaint in acutely ill
patients. You can always do a complete physical examination later.
Initial Management
Setting: emergency department
Diagnostic/Therapeutic Plan
Test Results
Assessment
The presence of elevated serum protein/albumin ratio in the setting of pathologic fracture
raises concern for multiple myeloma. In addition, the patient has symptoms in other bony areas
Plain film of the left shoulder
BUN, creatinine
Total protein/albumin
LFTs
CBC
Serum calcium
Fracture of proximal humerus with multiple lytic lesions
BUN, creatinine: 28, 2.0 mg/dL
Total protein/albumin: 9.0 g/dL (elevated), albumin 3.5 g/dL (normal)
LFTs normal
WBC 5,000/mm
3
(normal: 4,000–11,000/mm
3
), Hgb 11 mg/dL (normal 12–16 mgdL),
platelets 120,000/mm
3
Serum calcium 11.5 mg/dL (normal 8.5–10.2 mg/dL)
373

and evidence of renal failure. The most common presentation of myeloma is bone pain.
Basic Science Correlate
Mechanism of renal failure in myeloma:
Mechanism of hyperuricemia:
Why don’t myeloma bone lesions light up on a nuclear bone scan?
Further Management Plan/Results
IgG clogs up glomeruli
Uric acid damages tubules
Hypercalcemia toxic to tubules
Amyloid
Bence-Jones protein toxic to tubules
Purines and pyrimidines from plasma cell nuclei transform into uric acid
Technetium nuclear isotope is deposited by osteoblast. Myeloma lesions are purely
lytic
Normal saline bolus
1.
Pamidronate
2.
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Presence of monoclonal spike immunoglobulin
G: 6 g/dL
Positive for Bence-Jones protein
Multiple lytic lesions in the ribs and vertebrae
35% plasma cells
Elevated
Discussion
The patient requires an orthopedic procedure to correct the fracture. Radiation therapy to the
arm may be required for pain control. Ideally, radiation therapy should be limited initially so
that chemotherapy is not compromised by the depletion of bone marrow reserves.
There are numerous treatment options for chemotherapy and there is no one clear “best
choice.” Options are a combination of 2 or 3 of the following, such as bortezomib, prednisone,
or melphalan when age >70. Combination regimens, including other alkylating agents,
vincristine, doxorubicin, and prednisone, may yield a more rapid response with greater
adverse effects. Hypercalcemia should be initially managed with hydration and
bisphosphonates.
Multiple myeloma is primarily a disease of older patients. An elevated monoclonal
immunoglobulin spike characterizes it. It is differentiated from the more common monoclonal
gammopathy of unknown significance by the height of the M spike (>3 g protein), the presence
of bony lesions, or marrow infiltration with >10% plasma cells.
SPEP3.
Urine protein electrophoresis4.
Skeletal survey5.
Bone marrow6.
Beta 2 micro globulin7.
375

Multiple myeloma most commonly presents as bone pain involving the ribs and back. Bony
lesions are caused by infiltration of plasma cells and activation of osteoclasts that are
responding to a hormonal factor made by the myeloma cells. Bony lesions are almost
exclusively lytic, so bone scans are usually negative. Plain films diagnose bony lesions.
Hypercalcemia is often a problem because of mobilization of calcium from bone. Patients may
also suffer from cord compression because of involvement of vertebrae.
Patients with multiple myeloma are at increased risk for bacterial infections because of
associated diffuse hypogammaglobulinemia. This is caused by suppression of antibody
production aside from the monoclonal spike.
Staging relates to the presence of anemia, the height of the M spike, the presence of
hypercalcemia, and the degree of skeletal involvement. These, and beta-2 microglobulin, act as
prognostic indicators.
Multiple myeloma is usually responsive to but not curable with standard chemotherapy. A
variety of regimens involving steroids, alkylating agents, and thalidomide-derivatives have
been developed. Combination regimens usually lead to a more rapid response without
improving survival. Bortezomib is a proteasome inhibitor which is commonly used.
Once a patient is moved to a minimal disease state without symptoms, he may be observed until
problems later arise. Bony lesions can be treated with radiation therapy for pain control.
Autologous stem-cell transplantation is used in those age <70 in an attempt to drive the disease
into deeper remission.
Follow-up Management and Prevention
Surgical repair of humerus
Chemotherapy for myeloma with prednisone, melphalan, and possibly bortezomib initially
(in older patients for palliation)
Thalidomide or lenalidomide
376
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Basic Science Correlate
What is the mechanism of massive volume depletion in hypercalcemia?
How come hemolysis does not cause hyperuricemia?
How come autologous stem cell transplantation can be done at a much older age (up to
age 70) than allogeneic transplantation?
CLINICAL PEARL
Autologous bone-marrow transplantation in those age <70
Pamidronate (bisphosphonates)
High calcium inhibits ADH effect on V2 receptors in collecting duct, causing
nephrogenic diabetes insipidus
Osmotic diuresis
Mature red cells have no nuclei and therefore no nucleic acids
No rejection with autologous transplantation
No graft versus host disease
Stem cells grow to mature cells faster
Much shorter duration of neutropenia
377

Multiple myeloma
Final Diagnosis
Multiple myeloma
Clinically: bone pain and fractures, infections, anemia
Protein electrophoresis: monoclonal spike (M protein)
M protein >3 g
Renal insufficiency
Urine: Bence-Jones protein
Plasma cells >10% in the marrow (usually >30%)
378
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Case 4
Chief Complaint
Enlarged lymph node in the neck
History and Physical Examination
A 25-year-old woman comes to the office with a history of enlarged lymph nodes in her
neck. The nodes are in the cervical area and have been enlarged over the last several
weeks. She denies fever or weight loss. She is not on any medication.
Physical examination of the eyes, ears, nose, and throat is normal. There are several 2- to
3-cm, firm, freely mobile lymph nodes in the right anterior cervical chain. The nodes are
not warm, red, or tender. The chest is clear and heart is normal without murmurs. The
abdomen is soft and non-tender with no hepatosplenomegaly. There is no peripheral
edema noted.
CCS NOTE
Each part of the physical exam takes 1 minute when ordered individually, except for the
HEENT and pelvic and rectal exams. For each of those, give 2 minutes.
Differential Diagnosis
379

Initial Management
Setting: outpatient
Diagnostic/Therapeutic Plan
Test Results
Assessment
Rapidly enlarging, firm, nontender, cervical lymph nodes suggest lymphoma. Adenopathy,
fevers, night sweats, and cough are also suggestive of lymphoproliferative disease.
Tuberculosis and Epstein-Barr virus should also be considered when these occur.
Hodgkin’s lymphoma
1.
Non-Hodgkin’s lymphoma
2.
Tuberculosis
3.
Epstein-Barr virus infection
4.
HIV
5.
Acute lymphocytic leukemia
6.
Sarcoidosis
7.
CBC
LFTs
Chest x-ray
Cervical lymph node biopsy (excisional biospy)
CBC normal
LFTs normal
Chest x-ray normal
Cervical lymph node biopsy: nodular sclerosis Hodgkin’s lymphoma
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