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Unusual Extramedullary Plasmacytoma: A Rare but Possible Cause of Lymphadenopathy in Chronic Lymphocytic Leukemia.

Chantepie SP, Cabrera Q, Mear JB, Salaun V, Lechapt-Zalcman E, Macro M - Case Rep Med (2015)

Bottom Line: CLL front-line therapy was ineffective leading to adenectomy and diagnosis of concomitant extramedullary plasmacytoma.Radiotherapy did not result in the disappearance of lymphadenopathy.Adenectomy should be performed in CLL cases to avoid misdiagnosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, Caen University Hospital, 14000 Caen, France.

ABSTRACT
Cervical bilateral lymphadenopathy is a frequent event during chronic lymphocytic leukemia (CLL) natural history. However, lymph node biopsy is generally not required as long as transformation into an aggressive lymphoma (Richter syndrome) is not suspected. We present here a rare case of CLL patient who developed progressive bilateral cervical lymph node and bilateral tonsillar hypertrophy. CLL front-line therapy was ineffective leading to adenectomy and diagnosis of concomitant extramedullary plasmacytoma. Radiotherapy did not result in the disappearance of lymphadenopathy. Adenectomy should be performed in CLL cases to avoid misdiagnosis.

No MeSH data available.


Related in: MedlinePlus

(a) PET-CT before radiotherapy showing bilateral abnormal uptake in cervical bilateral adenopathy and in right tonsil; (b) and (c) PET-CT 2 and 4 months after radiotherapy showing the persistence of FDG uptakes in the previous involved site.
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fig2: (a) PET-CT before radiotherapy showing bilateral abnormal uptake in cervical bilateral adenopathy and in right tonsil; (b) and (c) PET-CT 2 and 4 months after radiotherapy showing the persistence of FDG uptakes in the previous involved site.

Mentions: A 63-year-old patient with a history of CLL since 2003 was referred to the Caen University Hospital. Binet's stage was A, and Matutes score was 5. Immunophenotype revealed CD5/CD19/kappa positive CLL cells. He complained of a gradual increase in bilateral cervical lymphadenopathy which measured 10 cm right and 5 cm left with bilateral tonsillar hypertrophy. He did not mention weight loss or night sweat. He also had lymphocytosis (8.5 G/L) and an IgG kappa monoclonal protein (24 g/L). Fludarabine, cyclophosphamide, and rituximab therapy was started without any response on lymphadenopathy after 6 cycles. Four cycles of R-CHOP were also ineffective. Right cervical adenectomy and right tonsillectomy revealed extramedullary plasmacytoma with the same kappa light chain on histological examination (Figure 1) and in flow cytometry analysis compared to the initial circulating CLL clone. Flow cytometry plasma cells were CD38/CD138/CD56/kappa strong and negative for CD19/CD20/CD33/CD117/CD45. No CLL cells were found in nodes and tonsils. The bone marrow biopsy and bone marrow aspiration failed to detect any CLL residual disease or clonal plasma cells. PET-CT scan showed FDG uptake in the right and left cervical area, the base of the tongue, and the right tonsil (Figure 2(a)). Surprisingly, radiotherapy 40 Gy was ineffective (Figures 2(b) and 2(c)). Lymph nodes sizes eventually decreased with bortezomib and dexamethasone therapy leading to a very good partial response on monoclonal component and cervical plasmacytoma disappearance. The patient is still alive 3 years after treatment without any lymphadenopathy but with a low residual monoclonal component (2 g/L).


Unusual Extramedullary Plasmacytoma: A Rare but Possible Cause of Lymphadenopathy in Chronic Lymphocytic Leukemia.

Chantepie SP, Cabrera Q, Mear JB, Salaun V, Lechapt-Zalcman E, Macro M - Case Rep Med (2015)

(a) PET-CT before radiotherapy showing bilateral abnormal uptake in cervical bilateral adenopathy and in right tonsil; (b) and (c) PET-CT 2 and 4 months after radiotherapy showing the persistence of FDG uptakes in the previous involved site.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4508473&req=5

fig2: (a) PET-CT before radiotherapy showing bilateral abnormal uptake in cervical bilateral adenopathy and in right tonsil; (b) and (c) PET-CT 2 and 4 months after radiotherapy showing the persistence of FDG uptakes in the previous involved site.
Mentions: A 63-year-old patient with a history of CLL since 2003 was referred to the Caen University Hospital. Binet's stage was A, and Matutes score was 5. Immunophenotype revealed CD5/CD19/kappa positive CLL cells. He complained of a gradual increase in bilateral cervical lymphadenopathy which measured 10 cm right and 5 cm left with bilateral tonsillar hypertrophy. He did not mention weight loss or night sweat. He also had lymphocytosis (8.5 G/L) and an IgG kappa monoclonal protein (24 g/L). Fludarabine, cyclophosphamide, and rituximab therapy was started without any response on lymphadenopathy after 6 cycles. Four cycles of R-CHOP were also ineffective. Right cervical adenectomy and right tonsillectomy revealed extramedullary plasmacytoma with the same kappa light chain on histological examination (Figure 1) and in flow cytometry analysis compared to the initial circulating CLL clone. Flow cytometry plasma cells were CD38/CD138/CD56/kappa strong and negative for CD19/CD20/CD33/CD117/CD45. No CLL cells were found in nodes and tonsils. The bone marrow biopsy and bone marrow aspiration failed to detect any CLL residual disease or clonal plasma cells. PET-CT scan showed FDG uptake in the right and left cervical area, the base of the tongue, and the right tonsil (Figure 2(a)). Surprisingly, radiotherapy 40 Gy was ineffective (Figures 2(b) and 2(c)). Lymph nodes sizes eventually decreased with bortezomib and dexamethasone therapy leading to a very good partial response on monoclonal component and cervical plasmacytoma disappearance. The patient is still alive 3 years after treatment without any lymphadenopathy but with a low residual monoclonal component (2 g/L).

Bottom Line: CLL front-line therapy was ineffective leading to adenectomy and diagnosis of concomitant extramedullary plasmacytoma.Radiotherapy did not result in the disappearance of lymphadenopathy.Adenectomy should be performed in CLL cases to avoid misdiagnosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, Caen University Hospital, 14000 Caen, France.

ABSTRACT
Cervical bilateral lymphadenopathy is a frequent event during chronic lymphocytic leukemia (CLL) natural history. However, lymph node biopsy is generally not required as long as transformation into an aggressive lymphoma (Richter syndrome) is not suspected. We present here a rare case of CLL patient who developed progressive bilateral cervical lymph node and bilateral tonsillar hypertrophy. CLL front-line therapy was ineffective leading to adenectomy and diagnosis of concomitant extramedullary plasmacytoma. Radiotherapy did not result in the disappearance of lymphadenopathy. Adenectomy should be performed in CLL cases to avoid misdiagnosis.

No MeSH data available.


Related in: MedlinePlus