Sabato 27 ottobre 2012 - Pacini Editore

COmuNiCaziONi ORali
A case of intravascular large b-cell lymphoma
sharing western- and eastern type features
A. Vanzati1 , F. Moltrasio1 , M.G. Valente2 , G. Cattoretti1 ,
G. Isimbaldi2 1 Deparment of Pathology, Department of Surgical Science, Unviersità di
Milano-Bicocca, Milano, Italy and Azienda Ospedaliera San Gerardo,
Monza, Italy; 2 Department of Pathology, Azienda Ospedaliera San Gerardo,
Monza, Italy
A 72 years-old woman complaining with paresthesia and hypomobility
of legs, was admitted to our neurologic department 10
days after an hysterectomy was performed in another institution.
The initial remission, obtained after a multidrug treatment
including steroids and antibiotics, was followed by the intermittent
recurrence of the neurologic symptoms. Rapidly, pancytopenia
and low platelet count was noticed, a macrophageactivation
syndrome was suspected and a bone marrow biopsy
showed increased cellularity with large amount of histiocytes
but no image of hemophagocytosis. Subsequently the patient
was admitted to the medical department where ciclosporin
therapy was administrated with initial partial efficacy. Few days
later renal and lung failure with pulmonary thromboembolism
made the situation worse: a Guillan-Barrè syndrome rather than
a chronic inflammatory demyelinating polyneuropathy was hypothesized.
RMN was performed and showed medullary lesions
in D11, D12, L1, L5, S1 and S2 in addition to two asymptomatic
extracerebral masses located at the frontal and parietal lobe and
referred as meningiomas. The woman died shortly afterwards
and necropsy was performed.
Macroscopically, the lungs were markedly congested, the heart
was flaccid; there was splenomegaly and hepatic steatosis. No
lymph nodes enlargement we re noted. CNS examination, comprehensive
of the spinal cord, revealed no superficial changes. No
cutaneous lesions were noted.
Routine sampling were obtained from various organs, including
lymph nodes, standard samples of the brain regions (white and
grey matter), brainstem and spinal cord comprehensive of the
roots of the cranial and spinal nerves.
Microscopically, in almost all organs we noted small capillary
vessels, mainly arterioles, filled by large cells. The cells had
very scant cytoplasm, irregular nuclei with open chromatin and
prominent nucleoli. Their lymphoid nature was confirmed by
immunohistochemical reactivity for CD79a and CD20. CD3 was
negative. The microscopic examination of lungs, pancreas, kidney,
adrenal glands, heart, ovaries, liver, spleen, spinal cord and
brain revealed the presence of intravascular proliferation of these
cells. In the lungs, the intraluminal packed cells caused multiple
acute subpleural infarcts so a diagnosis of multiorgan failure in
intravascular large B-cell lymphoma (IVL) was rendered.
Particularly, the examination of CNS slides demonstrated both
intraparenchymal and meningeal localizations and the roots of the
spinal nerves presented multiple intravascular deposits of the disease
in the vascular system of the schwannian sheats. Bone marrow
was not involved by intravascular neoplastic proliferation.
IVL is a well recognized entity by WHO classification of tumors
of lymphoid tissue, that occurs in adult patient (1,2) . Clinically, two
forms are recognized: the western and eastern ones. Our case presents
incomplete features of the two forms: macrophage activation
(with or without hemophagocytosis), thrombocytopenia, involvement
of spleen, multiorgan localization with failure and rapid
course are more characteristic of the eastern form. The absence
Fig. 1.
Fig. 2.
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of hemophagocytosis and the lymphoma sparing the bone marrow
seems to not completely satisfy this hypothesis. On the other
hand, her first symptoms were neurological in origin, typical of
western form of IVL. This difficulty in classify our case correctly
underlines the ambiguity of the criteria on which the differentiation
of the two form of IVL is based. Recent studies tried to solve
this matter (3) , and hemophagocytosis, irrespective of geographic
origin, seems to be the key. In conclusion we are still far to understand
the nature of this disease and more extensive investigations
need to better define disease and its biological behaviour.
references
1 Nakamura S, Ponzoni M, Campo E. Intravascular large B-cell lymphoma.
In: WHO Classification of Tumours of Haematopoietic and
Lymphoid Tissues. 4 th ed. 2008:252-253.
2 Ponzoni M, et al. Definition, diagnosis, and management of intravascular
large B-cell lymphoma: proposals and perspectives from an
international consensus meeting. J Clin Oncol. 2007;25:3168-73.
3 Ferreri AJ, et al. Variations in clinical presentation, frequency
of hemophagocytosis and clinical behavior of intravascular lymphoma
diagnosed in different geographical regions. Haematologica
2007;92:486-92.