Case Studies

1
Case Studies

• Dr Martin Auger
• Consultant Haematologist
• Norfolk Norwich University Hospital

2
Norfolk Norwich University Hospital
3
SFLC assay at NNUH

• Beckman Coulter IMMAGE
• October 2006
• 500 patient-samples per year (and rising)
• gt90 from Dept Haematology, NNUH

4
SFLC assay at NNUHOctober 2006

• Indications
• - monitoring of light chain myeloma
• - monitoring of non-secretory myeloma
• - monitoring of AL amyloidosis
• - aid to diagnosis of light chain and non-
• secretory
  myeloma
• - aid to diagnosis of AL amyloidosis

5
PT 48yrs male

• Presented, 2004
• -Worsening tiredness and nausea
• -Back pain
• -Previously fit swimming instructor
• - Urea 14.2 mmols/l
• - Creatinine 543 umols/l
• - Corr Ca 3.02mmol/l Hb 10.1g/dl

6
PT 48yrs male

• Investigations, 2004
• -24hr urinary BJP 11.76 g/24hrs
• - no serum paraprotein
• - Bone marrow 90 plasma cells
• - Skeletal survey osteopenia
• wedge s T8,
  T9, T11
• small lytic
  lesions

7
PT 48yrs male
8
PT 48yrs male

• Diagnosis Light chain myeloma, with renal
• 
  failure
• - creatinine 213umol/l
• Treatment
• -July Sept 2004
• - CVAD x4 (Myeloma IX)
• -Nov 2004
• -PBSCT with melphalan 140mg/m2

9
PT 48yrs male

• Feb 2005
• -24 hr urinary BJP 0.05g/24hrs

10
PT 48yrs male
11
PT 48yrs male

• Myeloma progression, Jan 2008
• Myeloma IX Sub-protocol
• - Bortezomib
• - Dexamethasone

12

• SFLC can be used to monitor light chain myeloma
  and are probably easier and more accurate than
  24hr urine collections

13
BW 72yrs female

• Presented, March 2004
• - dysphagia
• - hoarseness
• - anorexia
• - weight loss
• - nodular skin rash
• - chest pain.

14
BW 72yrs female

• Systemic AL amyloidosis
• - gastro-intestinal tract (OGD)
• - heart
• - vocal cord
• - skin
• - bone marrow
• - liver/spleen involvement on SAP scan
• - no renal involvement!

15
BW 72yrs female

• Investigations, 2004
• - small IgGk paraprotein
• - no urinary BJP
• - bone marrow 4 plasma cells
• - skeletal survey no myeloma bone
• 
  disease
• - renal function, LFT, FBC normal
  

16
BW 72yrs female

• Treatment
• - June-Sept, 2004
• - Intermediate dose melphalan x4
• - October, 2004 March, 2005
• - CTD x5

17
BW 72yrs female
18

• SFLC ratio can be used to monitor response to
  treatment in systemic AL amyloidosis

19
RS 62yrs male

• Presented, May 2007
• - Generally unwell
• - Pain in back and ribs
• - GP found a paraprotein
• Referred Haematology Dept

20
RS 62yrs male

• Investigations, May 2007
• - Paraprotein IgGk 15.2g/l
• -Bone marrow 84 plasma cells
• - Skeletal survey generally osteopenic
• bones
• - Hb 10.6g/dl Corr Ca 3.45mmol/l
• Urea 12.5 Creatinine 188

21
RS 62yrs male
22
RS 62yrs male

• SFLC
• Free kappa 39,850 (Ref 3.3-19.4)
  
• Free lambda 11.8 (Ref
  5.7-26.3)
• kappalambda ratio 3,377 (0.26-1.65)

23
RS 62yrs male

• Treatment
• -May-Oct, 2007
• -CTDa chemotherapy x 7
• - Feb, 2008
• - PBSCT with HDM

24
RS 62yrs male
25
RS 62yrs male

• Very high SFLC levels may be seen in patients
  with a modest paraprotein and indicate more
  aggressive disease
• SFLC levels may give an earlier indication of
  response to treatment than paraprotein level

26

• High SFLC levels and their rapid reduction in
  response to therapy may define an aggressive
  subtype of myeloma with poor prognosis
• (Blood 2007110827-832)

27
MS 67yrs female

• Presentation in Spain, January 2006
• - acute onset inter-scapular pain
• - some numbness/weakness in legs
• - given steroids and one dose radiotherapy
• 
  to spine
• - transferred to Norfolk Norwich

28
MS 67yrs female
29
MS 67yrs female

• Investigation, Jan 2006
• - MRI destructive lesion T5 with some
• spinal cord compression
• - Spinal decompression/fusion
• - Histology of tumour plasmacytoma or
• 
  myeloma

30
MS 67yrs female

• Investigations, Jan 2006
• - IgG lambda paraprotein 5.5g/l
• - Full skeletal survey T5 lesion, nil
  else
• - Bone marrow biopsy 5 plasma cells
• - FBC, UE, Ca normal
• Diagnosis solitary bone plasmacytoma

31
MS 67yrs female

• Treatment, March 2006
• - Radiotherapy
• - 40Gy in 22 fractions to thoracic spine
• Fully recovered mobility and well

32
MS 67yrs female

• Re-presented, March 2007
• - pain right shoulder
• Radiology, April 2007
• -radiographs/MRI spine collapse C4 with
  encroachment of a mass on C4 nerve root

33
MS 67yrs female
34
MS 67yrs female

• Investigations, March 2007
• - FBC normal
• - UE normal
• - Calcium normal
• - Paraprotein stable

35
MS 67yrs female

• Radiotherapy, May 2007
• - 20Gy in 5 fractions to cervical spine
• - steroids
• Chemotherapy
• - melphalan/steroids 6 courses

36
MS 67yrs female
37

• SFLC can be used to assess patients with solitary
  bone plasmacytoma or myeloma, but in whom there
  is only a small paraprotein band
• SFLC may indicate a response to chemotherapy, not
  seen with paraprotein measurements

38

• Immunoglobulin free light chains and solitary
  plasmacytoma of bone
• (Blood 20061081979-1983)

39
AK 67yrs male

• Presented 2004
• - acute renal
  failure
• - anuric
• Past history hypertension

40
AK 67yrs male

• Investigations, 2004
• - renal biopsy kappa light chain
  staining
• in glomeruli
• no evidence of
• amyloidosis
• - serum free kappa light chains.
• no intact immunoglobulin
  band

41
AK 67yrs male

• Investigations, 2004
• - Bone marrow 17 plasma cells and
• plasmablasts
• - Skeletal survey generalised
  osteopenia
• multiple
  lytic lesions
• DiagnosisLight chain (kappa) myeloma with end
• stage renal failure and anuria

42
AK 67yrs male

• Haemodialysis t.i.w.
• Epo
• Treated with intermittent dexamethasone
• and continuous thalidomide
• Declined repeat bone marrows

43

• SFLC ratio can be used to monitor light chain
  myeloma in patients with anuric renal failure on
  haemodialysis

44
The Future of SFLC in Norwich

• Stop 24hour urines for BJP quantitation
• Screen all new patients with possible myeloma
• - MGUS risk stratification
• - myeloma risk stratification

45
(No Transcript)