Case presentations of 3 patients with WM
| Case . | . | . |
|---|---|---|
| Case 1 | WM patient with IgM-related peripheral neuropathy | A 67-year-old patient complained of numbness and tingling in his toes that extended above the ankles over several months. Exam revealed diminished pinprick sensation over the soles of his feet. An IgMλ monoclonal protein was discovered, with a serum IgM level of 640 mg/dL. A bone marrow (BM) biopsy showed 10% involvement with LPL, and the MYD88L265P mutation was present. Fat-pad biopsy with congo-red staining was negative. An anti-MAG IgM antibody screen was markedly positive with a titer >1:102 400. |
| Case 2 | Newly diagnosed WM patient in hyperviscosity crisis | A 42-year-old male presented with blurry vision and nosebleeds. Exam revealed retinal hemorrhages, adenopathy, and splenomegaly. Laboratories revealed a hematocrit of 18%, platelets of 50 000/mm3, and leukocyte count of 1500/mm3. Serum total protein was high prompting a workup that revealed an IgMλ monoclonal protein and serum IgM level of 12 400 mg/dL. CT scans showed bulky adenopathy, and a BM biopsy demonstrated 80% LPL involvement. MYD88L265P mutation was present. |
| Case 3 | Patient with refractory WM disease | A 46-year-old male was diagnosed with WM after presentation with fatigue and a hematocrit of 28.6%. BM biopsy showed 90% LPL involvement. CT scans were unremarkable, and serum IgM was 2780 mg/dL. Treatment with bortezomib, dexamethasone, and rituximab was started. After 3 cycles, no treatment response was observed. He then received 2 cycles of cyclophosphamide-based (CDR) therapy without response. Bendamustine was then initiated, and after 2 cycles, serum IgM and hemoglobin levels remained unchanged. Everolimus was then begun and stopped due to nonresponse and worsening blood counts. A repeat BM biopsy confirmed unchanged WM disease. |
| Case . | . | . |
|---|---|---|
| Case 1 | WM patient with IgM-related peripheral neuropathy | A 67-year-old patient complained of numbness and tingling in his toes that extended above the ankles over several months. Exam revealed diminished pinprick sensation over the soles of his feet. An IgMλ monoclonal protein was discovered, with a serum IgM level of 640 mg/dL. A bone marrow (BM) biopsy showed 10% involvement with LPL, and the MYD88L265P mutation was present. Fat-pad biopsy with congo-red staining was negative. An anti-MAG IgM antibody screen was markedly positive with a titer >1:102 400. |
| Case 2 | Newly diagnosed WM patient in hyperviscosity crisis | A 42-year-old male presented with blurry vision and nosebleeds. Exam revealed retinal hemorrhages, adenopathy, and splenomegaly. Laboratories revealed a hematocrit of 18%, platelets of 50 000/mm3, and leukocyte count of 1500/mm3. Serum total protein was high prompting a workup that revealed an IgMλ monoclonal protein and serum IgM level of 12 400 mg/dL. CT scans showed bulky adenopathy, and a BM biopsy demonstrated 80% LPL involvement. MYD88L265P mutation was present. |
| Case 3 | Patient with refractory WM disease | A 46-year-old male was diagnosed with WM after presentation with fatigue and a hematocrit of 28.6%. BM biopsy showed 90% LPL involvement. CT scans were unremarkable, and serum IgM was 2780 mg/dL. Treatment with bortezomib, dexamethasone, and rituximab was started. After 3 cycles, no treatment response was observed. He then received 2 cycles of cyclophosphamide-based (CDR) therapy without response. Bendamustine was then initiated, and after 2 cycles, serum IgM and hemoglobin levels remained unchanged. Everolimus was then begun and stopped due to nonresponse and worsening blood counts. A repeat BM biopsy confirmed unchanged WM disease. |