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