Hyperviscosity-related retinopathy and serous macular detachment in Waldenström’s macroglobulinemia: A mortal case in 5 years

High Concentration ◽

Serous Macular Detachment ◽

Macular Detachment ◽

Purpose: To present a 5-year mortal case of Waldenström’s macroglobulinemia-related retinopathy and serous macular detachment. Case report: A 63-year-old man, with unremarkable medical history, presented with bilateral decreased vision for 2 months. Fundus examination revealed bilateral scattered retinal hemorrhages, exudates, venous tortuosity, and serous macular detachment. Hematologic and biochemistry profiles showed pancytopenia with blood smear demonstrating erythrocyte rouleaux formation. Hyperviscosity syndrome was suspected and later Waldenström’s macroglobulinemia was diagnosed by bone marrow biopsy and high concentration of serum IgM. Plasmapheresis and subsequent chemotherapy were arranged. In spite of resolution of most retinal abnormalities, his visual acuity still showed no improvement with a persistent bilateral macular detachment. The patient then died 5 years after the diagnosis of Waldenström’s macroglobulinemia. Conclusions: Long-term toxicity of IgM to the retinal pigment epithelium may impede the resolution of the persistent serous macular detachment, resulting in an inability of recovery in his vision. Therefore, early diagnosis and timely reduction of serum paraproteins by plasmapheresis and chemotherapy is critical for preventing permanent damages to patients’ health and vision.

Long-term response to sequential hemibody radiotherapy in waldenstrom's macroglobulinemia

Journal of Clinical Apheresis ◽

10.1002/jca.2920070413 ◽

1992 ◽

Vol 7 (4) ◽

pp. 219-220

Author(s):

Peter Jacobs ◽

Lucille Wood ◽

Margaret Shuttleworth

Keyword(s):

Waldenström's Macroglobulinemia ◽

Term Response

Long Term Responses to Fludarabine and Rituximab in Waldenstrom’s Macroglobulinemia

Blood ◽

10.1182/blood.v112.11.3057.3057 ◽

2008 ◽

Vol 112 (11) ◽

pp. 3057-3057 ◽

Cited By ~ 1

Author(s):

Steven Treon ◽

Andrew Branagan ◽

Leukothea Ioakimidis ◽

Jacob Soumerai ◽

Christopher Patterson ◽

...

Keyword(s):

Myelogenous Leukemia ◽

Aggressive Lymphoma ◽

Unknown Primary ◽

Term Outcome ◽

Long Term Outcome ◽

Abstract Fludarabine and rituximab are commonly used in combination in the treatment of Waldenstrom’s macroglobulinemia (WM), though long term outcome of this regimen remains to be defined. We therefore examined the outcome of 43 WM patients treated on a clinical trial whose eligibility included < 2 prior therapies, and no previous nucleoside analogue or rituximab treatment. Therapy consisted of 6 cycles (25 mg/m2/day for 5 days) of fludarabine and 8 infusions (375 mg/m2/week) of rituximab over 31 weeks. 43 patients were enrolled with a median age of 61, and median prior therapies of 0. Responses were: CR (n=2); VGPR (n=14); PR (n=21); MR (n=4); for an overall and major response rate of 95.3% and 86.0%, respectively. At best response, median bone marrow disease involvement declined from 55% to 5% (p<0.00001), while serum IgM decreased from 3,840 to 443 mg/dL (p<0.00001), and hematocrit rose from 31.2% to 38.0% (p<0.0008). The median time to progression for all patients was 51.2 months, and was longer for untreated versus previously treated patients (77.6 vs. 38.4 months; p=0.017), as well as for those patients who achieved ≥ VGPR versus <VGPR (>88.3 vs. 36.9 months; p=0.049). Grade ≥ 3 toxicities included neutropenia (n=27); thrombocytopenia (n=7); pneumonia (n=6), including two patients who succumbed to non-PCP interstitial pneumonia; peripheral neuropathy (n=2); limbic encephalitis (n=1); hemolytic anemia (n=1). With a median follow-up of 40.3 months, we observed transformation to aggressive lymphoma (n=3); myelodysplasia (n=1); acute myelogenous leukemia (n=2); bladder carcinoma (n=1); and carcinoma of unknown primary (n=1) among 8 patients. The results of this study demonstrate that fludarabine and rituximab is an active regimen in WM, though short and long term toxicities need to be carefully weighed against other available treatment options.

Diagnostic Tools of Waldenström’s Macroglobulinemia – Best Possibilities for Non-invasive and Long-term Disease Monitoring

Klinicka onkologie ◽

10.14735/amko20172s81 ◽

2017 ◽

Vol 30 (Suppl 2) ◽

pp. 2S81-2S91 ◽

Cited By ~ 2

Author(s):

Kateřina Growková ◽

Zuzana Kufová ◽

Tereza Ševčíková ◽

Jana Filipová ◽

Michal Kaščák ◽

...

Keyword(s):

Diagnostic Tools ◽

Disease Monitoring ◽

Non Invasive ◽

Long-term responses to thalidomide and rituximab in Waldenstrom's macroglobulinemia

Journal of Clinical Oncology ◽

10.1200/jco.2007.25.18_suppl.8017 ◽

2007 ◽

Vol 25 (18_suppl) ◽

pp. 8017-8017 ◽

Cited By ~ 4

Author(s):

J. D. Soumerai ◽

A. R. Branagan ◽

C. J. Patterson ◽

Z. R. Hunter ◽

S. P. Treon

Keyword(s):

Response Rates ◽

High Serum ◽

Highly Active ◽

Best Response ◽

Major Response ◽

8017 Background: Rituximab is active in Waldenstrom's macroglobulinemia (WM), producing response rates of 40–50%. Lower response rates are observed among patients with the FcγRIIIA-158 FF polymorphism; high B2M (≥3.0 mg/dL), and high serum IgM levels (≥6,000 mg/dL). Thalidomide enhances rituximab-mediated ADCC of lymphoplasmacytic cells and produces response rates of 25% in WM. As such, we conducted this phase II study using thalidomide and rituximab in patients naïve to either agent. Methods: Intended therapy was: Weeks 1–52: Thalidomide (200 mg po qHS for 2 wks, then 400 mg po qHS) Weeks 2–5, 13- 16: Rituximab (375 mg/m2/wk) Twenty-five patients were enrolled, 20 of whom were previously untreated, with a baseline median age of 62 (range 44–86 yrs), BM involvement of 40 (range 5–80%), serum IgM of 3,670 (range 924–8,610 mg/dL), B2M of 2.6 (range 1.4–8.3 mg/L), Hct of 34.1 (range 23.6–42.6%). Results: Grade ≥2 toxicities to thalidomide included neuroparesthesias (n=11); somnolence (n=3); confusion (n=3); rash (n=2); tremors (n=2); bradycardia (n=2) and palpitations (n=1). Among patients experiencing neuroparesthesias, 10 demonstrated resolution to grade 1 (n=3) or complete resolution (n=7) at a median of 6.7 (range 0.4- 22.5 months). Dose reduction of thalidomide occurred in all patients and led to discontinuation in 11 patients. Twenty-three patients were evaluable. Responses among evaluable patients: CR (n=1); PR (n=15); MR (n=2); SD (n=1) for an overall and a major response rate of 78% and 70%, respectively. Median serum IgM decreased from 3,670 (924–8,610 mg/dL) to 1,590 (36–5,230 mg/dL) (p<0.001), while median hematocrit rose from 33.0 (23.6–42.6%) to 37.6 (29.3–44.3%) (p=0.004) at best response. With a median follow-up of 42+ months, the median TTP for all evaluable patients on study was 35 months, and 38+ months for responders. Overall response was associated with median cumulative thalidomide dose: CR/PR/MR (29,275 mg) vs. SD/NR (7,400 mg); p=0.004. Overall responses were unaffected by FcγRIIIA-158 polymorphism status (81% vs. 71% for VV/FV vs. FF); serum IgM (78% vs. 80% for <6,000 vs. ≥6,000 mg/dL); and B2M levels (71% vs. 89% for <3 vs. ≥3 g/dL); p=NS. Conclusions: Thalidomide in combination with rituximab is highly active and produces long- term responses in patients with WM. No significant financial relationships to disclose.

Multimodal imaging of bilateral immunogammopathy maculopathy associated to Waldenström’s macroglobulinemia

European Journal of Ophthalmology ◽

10.1177/11206721211039342 ◽

2021 ◽

pp. 112067212110393

Author(s):

Rim Bouraoui ◽

Khaled El Matri ◽

Yousra Falfoul ◽

Dalel Fakhfakh ◽

Fatma Mghaieth ◽

...

Keyword(s):

Multimodal Imaging ◽

Subretinal Fluid ◽

Diabetic Woman ◽

Fluid Accumulation ◽

Macular Detachment ◽

Intraretinal Fluid ◽

Aim: Our aim is to report a case with bilateral Waldenström’s macroglobulinemia (WM) associated maculopathy, assessed with multimodal imaging including swept source optical coherence tomography (SS-OCT) and OCT-Angiography (OCT-A). Methods: Observational case report. Case presentation: A 61-year-old diabetic woman with history of treated WM currently in remission, presented with progressive bilateral visual loss. Best-corrected visual acuity was 20/100 in the right eye (RE) and 20/200 in the left eye (LE). Fundus examination showed bilateral microaneurysms and retinal punctuate hemorrhages and a large macular serous detachment in the LE. There was no retinal ischemia on FA nor macular dye leakage. SS-OCT showed a significant schisis-like intraretinal fluid accumulation in the RE and a large prominent macular detachment with significant subretinal fluid accumulation in the LE. Retinal and choriocapillaris vascular densities were normal on OCT-A. Conclusion: Our case illustrated characteristic multimodal imaging findings in WM associated maculopathy such as schisis-like intraretinal fluid accumulation and angiographically silent serous macular detachment. OCT-A could non-invasively analyze macular vascular densities layer-by-layer, without noticing any vascular anomaly.