Page 453 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 22 ■ Lymphoid and Plasma Cell Neoplasms 437

Disorder Laboratory Diagnostic Criteria

Solitary plasmacytoma with All criteria must be met:

minimal marrow involvement ‡ 1. Biopsy-proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells

2. Clonal bone marrow plasma cells <10%

3. Normal skeletal survey and MRI (or CT) of the spine and pelvis (except for the primary

solitary lesion)

4. Absence of end-organ damage, e.g., CRAB, attributable to a lymphoplasma cell

disorder.

POEMS syndrome Both criteria must be met:

1. Polyneuropathy

2. Monoclonal plasma cell proliferative disorder

Any one of the three other major criteria:

1. sclerotic bone lesions,

2. Castleman’s disease,

3. elevated levels of VEGFA

Systemic AL amyloidosis Any one of the following six minor criteria:

1. Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)

2. Extravascular volume overload (edema, pleurl effusion, or ascites)

3. Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)

4. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora,

acrocyanosis, ushing, white nails)

5. Papilloedema

6. Thrombocytosis/polycythemia

All criteria must be met:

1. Presence of an amyloid-related systemic syndrome (e.g., renal, liver, heart, gastrointes-

tinal tract, or peripheral nerve involvement)

2. Positive amyloid staining by Congo red in any tissue (e.g., fat aspirate, bone marrow, or

organ biopsy)

3. Evidence that amyloid is light-chain-related established by direct exmination of the amy-

loid using mass spectrometry-based proteomic analysis or immunoeletronmicroscopy

4. Evidence of a monoclonal plasma cell proliferative disorder (serum monoclonal protein,

abnormal free light chain ratio, or clonal plasma cells in the bone marrow)

Reference: International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma, www.imwg.myeloma.org, October 29, 2015, based on
Rajkumar SV Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group revised updated criteria for the diagnosis of multiple myeloma. Lancet
,
Oncol 15(12):e538–e548, 2014.

* CRAB = hypercalcemia = serum calcium >0.25 mmol/L (>1 mg/dL) higher than the upper limit of normal or >2.75 mmol/L (>11 mg/dL), renal insuf ciency = creati-

nine clearance <40 mL/min or serum creatinine >177 µmol/L (>2 mg/dL), anemia = hemoglobin of >2 g/dL below the lower limit of normal, or a hemoglobin value
<10 g/dL, bone lesions—one or more osteolytic lesions on skeletal x-ray, CT, or PET-CT.

† Monoclonal gammopathy of undetermined signi cance

‡ Solitary plasmacytoma with 10% or more plasma cells is considered multiple myeloma.

ten ency tow r thro bosis, which y be ni este is IgG in the jority o p tients. Less requently IgA is seen,

by shortene co gul tion ti e, incre se f brinogen, n n r rely IgD is e onstr te .

incre se ctor VIII. Growing clinic l ccept nce o seru ree light ch in

Electrophoresis o seru usu lly e onstr tes the ss y h s ll but eli in te urine tests or Bence-Jones pro-

overpro uction o IgM (19S) ntibo ies. Electrophoresis tein in the i entif c tion o MM. Monoclon l ree light ch ins

(Figs. 22.19 n 22.20) o the seru or urine reve ls t ll c n occur either s p rt o n int ct onoclon l i u-

sh rp pe ks on the ensito eter tr cing; ense loc lize noglobulin or s single pro uct. Usu lly, these ree light

b n is seen in 75% o yelo c ses. A onoclon l seru k pp n l b ch ins re boun e to the he vy ch in

protein is etecte in 91% o p tients. T e type o ntibo y o the i unoglobulin. Pl s cells lso pro uce lw ys n