caso clínico hospital la fe valenciacl¡nico...• esplenomegaly and adenomegalia without...
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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