notes by dr Claudio Italiano
Lymphomas are divided into:
a) Hodgkin's lymphoma
b) Non-Hodgkin's lymphomas
They constitute a group of complex neoplasms that ultimately derive from the expansion of a cellular clone (cell line) in the neoplastic sense of a given population of lymphocytes, that is, of the cell lines in the body responsible for immunological defense. The clinical pictures are disparate because the cells of the immune system are distributed widely in the organism and therefore the organs involved will be multiple and, as well, multiple symptoms will be depending on the degree of malignancy of the lymphoma itself which depends, in last analysis, from the histological type of the involved cells, that is if they are more differentiated and, therefore, more similar to normal lymphocytes, the symptomatic picture will be less evident. This allowed, today the progress in understanding and defining the anatomy and physiology of the immune system and the possibilities offered by modern cytohistomorphological analysis (ie the study of cellular jambs in the laboratory) allow a good classification, diagnosis and treatment of the various forms of lymphoma.
Lymphomas affected more males, with 100 cases / 1000,000 inhabitants / year in the USA; the etiopathogenetic cause is unknown but, probably, at the base of the nature of the lymphoma there is a defect of the immune system, of immunoregulation and immunosurveillance, so much so that subjects affected by s. from hereditary immunodeficiency are most affected.
To this group of individuals belong also subjects suffering from collagen diseases, es. S. of Sjogren which can be associated with the appearance of LnH. Other epidemiological studies have shown association between cases of Burkitt's lymphoma in geographic areas, e.g. Tropical Africa and New Guinea, and Epstein Barr virus, virus of the herpesviridae family.
The lymph node affected by the disease presents a totally subverted
architecture, often without invasion of the capsule. There is a so-called "reactive"
cell population and Reed-Stenberg (R-S) cells and Hodgkin mononuclear cells,
which are also found in lymphadenitis as an infectious origin of lymphoma. R-S
cells or "spectacle cells" have a diameter of 15-45 microns and show multiple
or multi-lobed central nuclei with voluminous nucleoli; the chromatin is
compressed at the periphery, configuring a thickening of the nuclear membrane;
however, there are variants of the R-S cell, the L & H type with a plurylobed
nucleus and small nucleoli, the lavunar type, in which the cell has a clear
lacunar space, the pleiomorphic variant. The lymph nodes still exhibit a
variability in the fibrotic component with the frameworks ranging from nodular
sclerosis to the predominance of lymphocytes and to the reticular variety. The
origin of R-S cells is still unknown but it is thought that they derive from B
lymphocytes and are CD30 +; other AAs believe that in the nodular sclerosis
variety the origin may be T-lymphoid.
Patient with Sezary syndrome after appropriate
radiotherapy cycle centered on the left shoulder;
note the pigmented appearance of the dermis and
the de-epithelialized areas in the subscapular
region. The patient performed 2 years of
chemotherapy and subsequent autologous
bone marrow transplantation.
It is initially confined to a single lymph node site but is subject to total
blood circulation, with consequent spread to the liver, to the spleen of its
cells. Classically, the Ann Arbor symposium of 1977 admits a staging of the
affection limited to a single lymph node region or to multiple organs, on one
side and the other of the diaphragm depending on the staging:
The stage: a disease limited to a single lymph node region (I) or to a single
extraliphatic organ or site (IE)
Stage II: affection in two or more lymph node regions on the same side of the
diaphragm (II) or localized involvement of an extralymphatic organ or site
associated with the invasion of one or more lymph node regions on the same side
of the diaphragm
Stage III: affection in multiple lymph node regions above and sub-diaphragmatic
(III); may be accompanied by splenic involvement (IIISE), localized involvement
of an extralymphatic organ or site (IIIE) or both (III SE)
Stage IV: widespread involvement, disseminated with one or more extralymphatic
organs or tissues without invasion of lymph node stations.
The initial symptom is a completely indolent lymph node swelling, with slow,
almost imperceptible growth, sometimes with a variation in the volume of the
lymph nodes, which appear to be mobile, hard, stone, movable, indolent;
especially affected the laterocervical, axillary, inguinal, supraclavicular;
sometimes the onset may be represented by a lymphatic stasis by compression of
the lymphatic system by lumbosodal and / or iliac lymph node stations, with
edema of the lower limbs; the invasion of the spleen is highlighted with
enlargement of the organ and occurs in 45% of cases. Other extralonfonodal sites
are the pulmonary parenchyma, the pleura, the liver, the pericardium, the bone
tissue, the Central Nervous System. The patient is affected from the beginning
by fever with night sweats, weight loss and itching, fever is remitting and
undulating. The chest should be investigated for RX and may show enlarged
mediastinal and / or hilar lymph nodes; in this case the survey will be
completed with the CT and the RMN; a study of abdominal lymph node stations can
be performed using bipedal lymph angiography and CT scan to study pelvic,
para-aortic and thoracic lymph nodes. Invasion of the bone marrow will be
accompanied by an increase in alkaline phosphatase and a myelophysic syndrome;
laboratory * highlights a normochromic, normocytic anemia with hyposideremia and
hypothransferrinemia present in 1/3 of the cases. Sometimes hemolytic anemias
are present with negative Coombs test, leukocytosis with 12,000 GB / mm3.
Infectious complications: they are sustained by bacterial infections from
staphylococcus aureus, Klebsiella, enterobacter, pseudomonas aeruginosa, mycetes,
toxoplasma, viruses such as herpes zoster. Finally, there is the risk of a
second neoplasm in Hodgkin's course, for example. of acute non-lymphoid leukemia.
It is difficult, long and highly specialized and presupposes the stagging of the
neoplasm; in the initial stages it uses high-dose radiotherapy, 4000 rads with
extended field, e.g. "cloak" or "inverted y" used in the forms with a
para-aortic, iliac and inguino-femoral localization. In advanced stages, the use
of chemotherapy is necessary.
index hematology