+ B- cell Lymphoproliferative disorder for 8 years

A 75-years-old female with an 8 years diagnosis of mature B-cell proliferation disorder. At August, 1999 a CBC showed high WBC, and physical examination showed enlarged lymph nodes (cervical and axillaries). Peripheral blood revealed Hb: 14,8g/dl, WBC: 21,8 x109/l, lymphocytes 16 x 10⁹/l, platelets 241 x 10⁹/l and bone marrow trephine showed interstitial infiltration with small lymphocytes consistent with CLL stage A (Binet). In April, 2000 there was a significant increase in the lymph nodes and night sweats. PB immunophenotyping study showed a CD19/CD5 positive population consisted with B-CLL (Matutes score 4). The patient was treated with 6 cycles of COP, with evolution to pulmonary nodules and axillae bulky in 2001. From 2001 to 2004, a few cycles of Chlorambucil and 7 cycles of R-COP produced a good response. In April, 2005, the PB morphology and immunophenotype were consistent to atypical B-CLL (CD19, CD20, CD23, CD25, HLA-DR, IgM, IgD, CD79b, CD38, and sKappa,positive and CD5 negative), and PB karyotype showed no clonal abnormalities in 20 metaphases. In August, 2005, there was an increased in the number of lymph nodes and Rituximab and Fludarabine was started. The PB counts showed: Hb: 13g/dl, WBC: 69,6 x 109/l, lymphocytes: 62 x 10⁹/l, platelets: 145 x 10⁹/l. Inguinal lymph node biopsy showed diffuse large B-cell Lymphoma , Ki-67: 70%, cyclin D1 -, CD20 +, BCL2 +. From December, 2005 to April, 2006, 6 cycles of R-CHOP showed no response. From October, 2006 to May, 2007, regression of lymph nodes and clinical improvement was done with 6 cycles of MiCEP. At this time, cytogenetics and immunophenotyping studies of bone marrow were performed.

75 yrs

F

+

+

+

-

5,4

13,6

169

3,35 x 10⁹/l (lymphoid cells)

28% of lymphoid mature cells

B-cell Lymphoproliferative disorder (Atypical CLL) with evolution to diffuse large B-cell Lymphoma. Atypical CLL.

25% of total bone marrow cells are positive : CD20++, CD22+, CD25+, CD38, CD79b++, HLA-DR, sIgM, sIgD e sKappa ++.

not done

Inguinal Lymph node biopsy (August, 2005): Diffuse large B-cell Lymphoma, Ki-67: 70%, ciclina D1 -, CD20 +, BCL2 +.

not done

B-cell Lymphoproliferative disorder (Atypical CLL) with evolution to diffuse large B-cell Lymphoma.

(07-2007)

wide previous history (long previous history of chemotherapy), no chemotherapy after July, 2007

not applied

-

-

A

09-2007

2

bone marrow cells

72 hours with and without TPA (o-tetradecanoyl phorbol-13-acetate)

G

47,XX, t(1;6) (p35;p25),+12[13]/46,XX[7]

not done

not done

not done

In 2005, the Belgian group described the t(1;6)(p35.3;p25.2) in 8 patients with unmutated B-CLL. As in this case, this rare cytogenetic entity has been described in typical or atypical CLL (8/8 cases), with evolution to diffuse large B-cell Lymphoma (3/8 cases); trisomy 12 been a common additional abnormality (3/8 cases).