Caso clínico Hospital La Fe ValenciaCl¡nico...• Esplenomegaly and adenomegalia without...
Transcript of Caso clínico Hospital La Fe ValenciaCl¡nico...• Esplenomegaly and adenomegalia without...
Reunión Club Linfomas 2013
Caso clínico Hospital La Fe Valencia Empar Mayordomo-Aranda1, Enrique Jiménez-Herrero1, Blanca Boluda2,
Amparo Sempere 2, Santiago Montes3, Miguel Ángel Piris3,
J.Francisco Vera-Sempere1
Clinical Case and Medical History
• 17ys old girl with lymph node enlargement
• With 4 was diagnosed of primary immune
trombocitopenia (another institution)
• Controlled with only sporadic treatment
(gammaglobulines) untill 2009
• Every Bone marrow biopsy:
• Megacariocitic hyperplasia
• Madurative detention in white serie
• No blasts
Bone marrow Aspirate
Clinical Case and Medical History
• 2009: abdominal pain + trombopenia: treatment
with corticoides and gammaglobulines (during 1
year)
• Bone marrow aspirate:
• Megacariocitic hyperplasia
• Madurative detention in white serie
• No blasts
• Persisting trombopenia + leucopenia +
splenomegaly: splenectomy.
Splenectomy Splenic smear
•Erithroid and mieloid
precursos
•Scattered lymphocites and
plasma cells
•Normal immunophenotype
•No clonality
Splenectomy Histopathology
Clinical Report
• Trombopenia + neutropenia: treatment with
Rituximab
• No sintoms during a year
• Lymph nodes enlargement
• Hemograme:
• Hemoglobine 12.9 g/dL
• Platelets 38 x109/L
• Leucocytes 10.5x109/L (neutrófilos 7.4 x109/L,
linfocitos 1.7x109/L)
• Biochemestry: LDH 700 U/L
Cervical Lymph node exeresis
Cervical Lymph node exeresis
Cervical Lymph node exeresis Cervical Lymph node exeresis
CD 3 CD 79a
CD 20 PAX5
CD 79a
CD 138 Plasma cell
CD 56
Lambda Kappa
CD138 Plasma cell
CD 5
CD 23
CD 3
CD 43
CD 23 CD 43
CD 20 CD 5
IgG
IgM IgD
IgA
Diagnosis
B Small-Cell Lymphoma with extensive
plasmocitoid differentiation:
•Nodal Marginal zone lymphoma
•Lymphoplasmocitic lymphoma
•Autoimmune lymphoproliferative syndrome
• Cytomorphology and cytometry results
• To Send the case in consultation
Cytomorphology
Flow Cytometry
Flow Cytometry
Flow Cytometry
Immunophenotype
• 55% of B cells CD19 positive:
• 40% policlonal lymphocytes
• 2%: CD19+/CD20++/CD38- with high SSC kappa
• 13%: CD19+/CD20-/CD38++/CD138- kappa
• Molecular Biology:
• IgH Rearrengement
• c-myc and bcl-2 negative
Bone marrow biopsy
Diagnosis: nodal marginal zone lymphoma
• Lymphadenopathy without extranodal involment
• Marginal zone expansion with folicular colonization
• Eosinophiles, monocytoide B-cell, plasma cells
• CD20, CD43+/-, CD5-, CD23-, IgD+/-
• Paediatric
• Localized at the diagnosis
Diagnosis: Lymphoplasmocitic Lymphoma
• Lymphadenopathy with small lymphocytes + plasmacytoid lymphocytes + plasma cells
• Bone marrow involvement
• Paraprotein IgM
• Waldeström macroglobulinemia
• Autoantibodies
• Dilated sinuses, architectural destruction, histiocytes , plasma cells
• CD20, CD43-, CD5-, CD23+/-, IgD-, CD38
But IgG
Autoimmune lymphoproliferative syndrom
• Defectous apoptosis in lymphoid cells
• Increasing double negative αβ lymphocytes (CD4/CD8-)
• Esplenomegaly and adenomegalia without
infection/malignancy
• FAS mutation
• Other mutations:
• Caspasa 10 y 8
• NRAS y KRAS
• High levels of: IL-10, Fas ligand soluble, IL-18, vitamine
B12 and IgG.
• EBV +
• Other morphology
Diagnosis:
• Lymphoplasmocytic lymphoma
• Taking home messages:
• Difficult diagnosis with wide expectrum of
morphological features
• It is not necessary to be associated to an increased
IgM
• If any doubt: the diagnosis should be a small B cell
lymphoma with plasmacytic diferrentiation and a
differential diagnosis provided. (WHO, 2008)
Thank you