Waldenström's Macroglobulinaemia

Bruised

Step 1: View clinicals

A 49 year old woman presents with upper abdominal pain, intermittent nausea and vomiting, malaise, and night sweats for 3 weeks, in association with a 6 kg weight loss. She also experienced easy bruising during the same period. There was no history of epistaxis, hematemesis, melena, or other bleeding manifestations. Her medical, surgical, and drug histories are unremarkable. There is no family history of bleeding disorders. She does not smoke, drink or use recreational drugs. Her ESR is 31 mm/1h.


Step 2: Order all relevant investigations

Complete Blood Count + Peripheral Smear

WBC/DC: 12,900 /mm3 (4,500-10,500) Lymphocytes: 9,900 /mm3 Hb: 10.3 g/dL (10.5-18) Hct: 37% (36-44) RBC: 4.1x10^6/ml (4.0-4.9) Platelets: 82x10^3/mL (150-450x10^3) The peripheral smear shows rouleaux formation, with atypical lymphocytes and plasmacytoid cells.

Liver Profile

Total Bilirubin: 1.1 mg/dL (0.1-1.2) AST: 38 U/L (<35) ALT: 37U/L (<35) ALP: 142 U/L (33-131) GGT: 78 U/L (0-51) Total Protein: 9.3 g/dL (6.0-8.3) Albumin: 2.9 g/dL (3.2-5.0) INR: 1.2 (0.8-1.1) LDH: 315 U/L (56-194)

Bone Marrow Aspiration + Biopsy

The bone marrow aspirate reveals a population of small, atypical lymphocytes admixed with normal cells. H&E staining of the trephine biopsy specimen demonstrates large intratrabecular lymphoid aggregates; immunostaining reveals the lymphiod cells to be CD20 positive and CD10 negative. Flow cytometry shows 77% of the lymphoid cells to be CD19, CD20, CD79b and cytoplasmic kappa positive, and negative for CD5, CD10, CD38 and CD138.

Serum Protein Electrophoresis

Serum electrophoresis reveals a large IgM component (>30 g/L), along with a a mild increase in serum IgG concentrations. Immunofixation reveals a monoclonal band which fixes anti-IgM and anti-kappa. Bence-Jones proteins are not detected.


Step 3: Select appropriate management

Plasmapheresis
Rituximab
Cyclophosphamide
Allogenic Stem Cell Transplantation


Score: ★★☆