Lymphomas: Marginal zone, Cheato variant, follicular, mantle, sezary/Mycosis fungoïdes, granular lymphocyte lymphocytosis
Introduction.
After eliminating a reactive lymphocytosis (and the rare situation of lymphocytosis polyclonal with Binucleate lymphocytes), the first diagnosis to be evoked in an adult (chronic) lymphocytosis is that of chronic lymphoid leukemia (l LC):
LLC in its classical cytological form (80% of cases), = monotonous hyperlymphocytose with small mature lymphocytes, with numerous bursting cells (Gumprecht shadows);
Or LLC in a cytological variety (20% of cases): mainly the morphologically atypical LLC, more rarely the mixed LLC with small and large lymphocytes or the Prolymphocytoïde LLC.
In all cases the diagnosis of LLC is confirmed by the Immunophénotype and the score of Matutes (score 5 or 4).
However, either because the lymphocytic morphology on smear is peculiar either because the score of Matutes is < 3 or is a lymphoproliferation T, the diagnosis of LLC is questioned and the hypothesis of the "dissemination of a "Mature cell lymphoma" should be evoked. The clinical presentation of these patients is either stackable to that of an LLC (Asymtomatique or not) or immediately suggestive of lymphoma. Morphology and Immunophénotype allow to quickly orient the diagnosis.
The WHO 2016 CLASSIFICATION of B-and T-cell malignant LYMPHOPROLIFÉRATIONS is included in another DOCUMENT.
are to know:
Chronic lymphocytic leukemia See item "LLC".
Prolymphocytaire leukemia B, exceptional See item "LLC".
Hairy leukemia See the item "Hairy Leukemia".
Lymphoplasmocytaire lymphoma See the item "Macroglobulinemia's disease".
Lymphomas of the marginal zone
Follicular lymphoma
Mantle lymphoma
Prolymphocytaire leukemia T, exceptional See item "LLC".
T or NK proliferations with large granular lymphocytes
Proliferations with Sezary cells (Mycosis fungoïdes, Sezary Syndrome)
1. Lymphomas of the Marginal Zone (LZM).
Represent about 12% of all NHL and occur in adults (median around 60 years).
Within the WHO CLASSIFICATION 2016, we find:
* Splenic marginal zone lymphoma (20%; variable volume splenomegaly and sometimes adenopathies)
* Lymphoma of the Extraganglionnaire marginal zone of lymphoid tissue associated with mucous membranes (MALT; 70%)
* Lymphoma of the marginal ganglionic zone (< 10%; ganglionic infiltration, but neither splenomegaly nor MALT-type infiltration)
* There are also three provisional entities:
-unclassifiable leukemia/lymphoma Splenic B
-Small cell lymphoma B diffuse red pulp splenic
-Hairy leukemia variant
1.1. MALT lymphoma (LZM EXTRAGANGLIONNAIRE).
Adult disease (median around 60 years), a little more often a woman.
Develops in organs initially lacking lymphoid follicles, but which will accumulate B lymphocytes following chronic antigenic stimulation or autoimmune disease. Lymphocytes acquire genome anomalies and gradually lose their dependence on antigenic stimulation; It follows a histological transformation.
Stimulation by bacterial antigens, activating the NF-KappaB pathway: According to the organs affected:
Stomach: Helicobacter's responsibility in 90% of cases
Eye and eye Annexes: responsibility of Chlamydia Psittaci
Skin: Responsibility of Borrelia burgdorferi
Intestine: Responsibility for Campylobacter Jejuni
Auto-immune Diseases:
syndrome, where the risk is 44 faith s + High to develop a MALT of the salivary glands,
Hashimoto's thyroid, where the risk of developing a thyroid gland MALT is increased by 70 times.
PTS are clinically present at the IE stage.
Two types of LZM malt are classically distinguished: gastric malt (1/3 malt); A variant is called IPSID (immunoproliferative small intestine disease)
Non-gastric malts (other locations)
Diagnosis.
Histology. Lymphomateuse infiltration around B-reaction follicles (cells of varying appearance: lymphocytes, Lympho-plasma, centrofolliculaire Sometimes, or monocytoïde allure).
CBC. No anomaly (including: no lymphocytosis)
Immunophénotype. M-Type surface Ig, more rarely A or G. CD5-, CD23-, CD20 +, CD43 +/-
Cytogenetic. Anomalies in 1/3 of cases. Specific translocations to certain malts:
Stomach and lung: t (11; 18) (BIRC3-MALT1) or T (1; 14) (BCL10/IgHV)
Lung, skin, salivary, eye: t (14; 18) (IgHV/MALT1)
Trisomies (+ 3; + 18) inconstant and not very specific.
Treatment. A suitable and prolonged antibiotic treatment is effective in more than 80% of malt (e.g. gastric malt = IPP + Clarythromycine + amoxicillin). Otherwise: localized radiotherapy or rituximab and Monochemotherapy (Chloranbucil). Survival at 5 years > 85%.
1.2. Lymphoma of the splenic marginal ZONE.
Rare disease (2% of B lymphomas) and usually indolent (over 50 years, and especially the 60-70-year-old woman)
Lymphocytic blood release is more often indicative than splenomegaly
Impact: Remains to be evaluated in France. Its incidence is estimated to be 0.13/100000 in the United States and the frequency of all malignant lymphomas not Hodgkin is 0.6%.
Clinical Situation.
The disease is discovered most often following an anomaly of CBC: Leukocytosis (Hyperlymphocytose), anemia and/or thrombocytopenia (these last 2 related more to sequestration splenic than medullary infiltration)
Splenomegaly, rarely troublesome, is present in > 90% of PTS, discovered from the diagnosis or that will appear later (can become monstrous).
Association with hepatitis C in > 20% of cases (HCV-E2 glycoprotein interacts with B-lymphocyte CD81, activating BCR: This causes proliferation of B cells).
A particular form of this lymphoma is associated with hepatitis C and a type 2 (mixed) cryoglobulinemia.
Autoimmune events are identifiées in 15% of cases: primary biliary cirrhosis, rheumatoid arthritis, PTI, Ahal, cold agglutinins disease, circulating anticoagulants, acquired Willebrand disease, acquired angioedema In laC1-esterase inhibitor
A serum monoclonal protein of IgG or IgM type is rtrouvée in one-third of the cases (usually < 50 g/L)
Biological Aspects.
Infiltration of the follicles of the white pulp splenic by lymphocytes B memory, in the marginal zone. The origin is post-centrogerminative in 70% of cases (IgVH mutated).
[However, IgVH is not mutated in 30% of cases, and it is siggéré that the differentiation pathway is specific and does not imply transit within the germ centre]
CBC.
Frequent moderate anemia and/or thrombocytopenia: mainly by hypersplenism.
Inconsistent lymphocytosis, but can reach or exceed 30 G/L.
Lymphocytic morphology: either banal and +/-close to that of the lymphocytes of an LLC (often without, but sometimes with excess of exploded cells (shadows of Gumprecht),
is banal but with QQ lymphocytes to the nucleus monocytoïde (ie +/-horseshoe)
Either with a small number of urban lymphocytes
> > 20% of the city lymphocytes = splenic lymphoma with City lymphocytes. See below.
Immunophénotype. Non-low expression IgS (= N, sometimes high).
CD5-CD23-CD43-CD10-with Matutes score < or = 2.
Strong expression of CD180, while it is weak in the LLC and the lymphoma of the mantle
However: positivity of CD5 in 20% of cases, requiring the removal of lymphoma from the mantle, or even an LLC.
Lack of expression of IRTA1 (which is expressed in MALT)
Biochemistry. IgM or IgG peak in 1/3 cases (up to 30-50 g/L)
Cytogenetic. Anomaly (s) in 80% of cases.
Total or partial Down syndrome (3q): 30-80% of pts
12p Gain: 20% of pts
Anomalies considered typical of LZM: deletion or translocation involving 7q32 (30% of pts)
T (11; 14) (q13; q32) in 10-20% of cases.
Molecular biology. Mutation of NOTCH2 in 20% of cases, in the NF-KB pathway in 35% of cases, mutation of KLF2 in 30% of cases. No mutation BRAF V600E
Histology.
Splenomegaly. Usually the spleen weighs > 400-500 g; A lower weight is very rare in the LZM
BOM: sine infiltration rather characteristic (often associated with interstitial and nodular infiltration). Not totally specific
Treatment.
Three negative risk factors: Hb < 12 g/DL, LDH > N, albumin < 35 g/L (from 88% to 50% survival at 5 years if 0, 1 or > 1 risk factor).
It is only treated in cases of severe cytopenias and/or symptomatic splenomegaly.
The splenectomy corrects the cytopenias but not the blood and medullary lymphocytosis.
Then: Rituximab + chemotherapy.
For forms with HCV: the treatment of HCV infection may result in the remission of lymphoma.
Risk of transformation in large-cell diffuse NHL = 10%
Splenic lymphoma with Urban lymphocytes
Rare variety of splenic lymphoma of the marginal zone, with variable volume splenomegaly, observed mainly in older PTS (> 70 years) and usually without autoimmune manifestations, characterized by the presence in the blood of at least 20% of lymphocytes Villous.
Often indolent, with prolonged survival.
CBC. Anemia and inconstant (if not moderate) thrombocytopenia; Lymphocytosis inconstant (does not always reach 5 G/L) with > 20% of urban lymphocytes
Urban lymphocytes: round nucleus with condensed chromatin, basophile cytoplasm and villus with 1 or 2 poles, but sometimes also on the whole membrane.
Other related cellular aspects can be found:
-Lymphoplasmocytoïdes cells
-Larger cells with Monocytoïde core.
Villous
Remarks
-Contact of Ly Villen a few hours with EDTA makes the villus disappear.
-The presence of a small number (1-2%) of urban lymphocytes (villus with 1 pole of the cell, rather discreet) is occasionally observed:
In the healthy subject or in the NNé – infant, and in some cases of LLC or NHL coat
Differential diagnosis.
Lymphoplasmocytaire lymphoma. sometimes difficult Discrimination; Mutation of MYD88 L265P, absent in the LZM
Mantle lymphoma (for LZM CD5 +). Morphology, expression of cyclin D1/BCL1, SOX11 negativity and absence of T (11; 14) usually eliminate the NHL coat
The provisional entities within the LZM (see below). But these entities are rare
Sometimes the lymphocytosis B monoclonal
The malts scattered. Clinical history is important.
1.3. Lymphoma of the Ganglionic marginal ZONE.
Rare lymphoma and by definition without splenomegaly or impaired MALT.
Multi-node disease (infiltration of variable topography), often at stages III or IV at the outset, difficult diagnosis (no specific markers).
In > 80% of cases: IgVH mutated.
Possible but not very frequent blood and medullary release.
Serum monoclonal Ig Peak in 10% of cases.
No special scytogénétiques anomaly, except a high frequency of trisomies: + 3, + 7, + 12, + 18, or amplifications (1q, 3q, 7p). But no loss in 7q.
Chemotherapy treatment. Overall survival = 65% at 5 years.
1.4. Hairy Leukemia variant: Who provisional entity 2016.
Rare disease; Median age of patients > 70 years.
Splenomegaly is present in 85% of the cases and contrasts with the scarcity of hepatic (19%) and ganglionic (15%) impairment.
Several differences with hairy leukemia: anemia and thrombocytopenia are observed in 29% and 43% of cases
No Monocytopénie,
No neutropenia,
Leukocytosis (sometimes 30 G/L) with excess of urban contour cells but with a net nucleolus, a nucleus sometimes convoluted or bilobed (intermediate morphology between prolymphocyte and Tricholeucocyte); Absence of resistant tartrate acid phosphatase,
Different Immunophénotype: CD123, CD25, and annexin 1 are negative;
CD103 and CD11c are positive (phénoptype close to that of Ly Villen)
Histology. Rate: Diffuse seepage of red pulp with atrophy of white pulp (classical tricholeucocytose ID); BOM: quasi-exclusive sine infiltration
Cytogenetic and molecular Aspects.
-some cases described with 14q32 or 8q24 anomalies,
-Partial or complete 17p deletion (1/3 of cases), coupled with poor prognosis (Hockley et al, Leukemia 2011)
-No mutation of BRAF
-Mutation of MAP2K1 in 50% of cases (code for MEK1, downstream of BRAF in the cell activation pathway) [but also in almost all of the few cases of leukemias to Cheato negative BRAF that use the region IgVH4-34 (like the Cheato variant)].
Treatment. Splenectomy often effective. Median survival: 9 years (42% of patients die from non-NHL-related causes).
Attention: In the literature some authors do not differentiate between hairy leukemia in its hyperleucocytaire form and hairy (WHO) variant leukemia. Moreover it is described a form "Japanese of leukemia to Hairy", which approximates the variant form, but of evolution more indolent.
1.5. Diffuse lymphoma of the spleen Red Pulp (LDPRR): Who provisional entity 2016.
Rare entity identified in 2002; Age patients > 60 years old
Moderate Lymphocytosis (75% of cases)
Anemia, thrombocytopenia neutropenia: Rare.
Morphology of the cells AN: close to that of SLVL (but sometimes there are cells + large and distinctly nucléolées)
An immunological score based on five markers (1 point if CD76 +, si CD27-, if CD38-, 1point si fluorescence ratio of CD11c and CD22 (= RFI)).
LDPRR: score = 3 to 5; LSZM: score = 0 to 2.
[QQ cases reported with CD5 +].
Cytogenetic and molecular anomalies not yet well characterized.
2. Follicular lymphoma.
Common lymphoma (25% of all NHL), consisting of lymphoid cells from the centre of the follicle.
Discovered in adults (exceptional cases before age 20): median of occurrence = 55 years.
Frequent tumor Syndrome, with adenopathies and splenomegaly.
Medullary releases (quasi-constant and M.E.E. by molecular biology; in 50% of cases in cytology/histology), Blood (5-10% of cases), and in various organs from diagnosis, although the disease is often not symptomatic of diagnosis.
Histology.
The ganglionic architecture is disturbed but it still has pseudo-follicular structures, sometimes mixed with diffuse seepage zones.
The cells occupying these pseudo-follicles are of 2 types, large (centroblasts) or small (small centrofolliculaires cells), and their respective numbers determine various grades:
Grades 1 and 2: No or few large cells;
Grade 3 A: Many large cells but small cell persistence;
Grade 3 B: Majority of beaches made up solely of centroblasts.
Appearance in the blood.
Blood release in 5-10% of cases: sometimes moderate (QQ cells found in morphology or CMF), or in high Nb (with lymphocytosis > 4 G/L, which can exceed 50 g/L).
The follicular NHL without blood release is more common in humans, before 60 years, with thrombocytopenia often associated.
Cases with moderate release are more frequently women, less than 60 years of age, more often thrombopéniques (thrombocytopenia related to splenomegaly).
The presence of abnormal cells does not necessarily require specific alarm of lymphocytes (or "blasts? ") on CBC automata. However, parameters of research on some automatons indicate the presence of an abnormal lymphoid clone in situations of high leukocytosis.
Small centrofolliculaires cells (blood; GEMS).
Most frequently observed cells.
Close to the size of a small lymphocyte,
N/C ratio close to 1: no visible cytoplasm.
Dense, mature chromatin core, often split from a furrow: sometimes in Coffee bean.
1follic
Centroblaste (blood; GEMS).
Close to that of a lymphoblaste.
Irregular contour core; Nucleoli (2 to 5) are often arranged on the periphery of the nucleus.
Cells rarely visible in the blood (sometimes in the bone marrow, constantly on the cytoponctions ganglion).
Centroblaste
Immunophénotype (see corresponding document).
Cells B CD5-and CD 43-
Expression of CD10 by small and large cells in 80% of cases). However, the expression of CD10 is negative on blood centrofiolliculaires cells in 1 of 5 cases, even though this CD10 is clearly expressed in the ganglion
Coexpression CD10 +/CD38 + frequent
Cytogenetics and Molecular Biology.
Quasi-constant presence of translocation T (14; 18) (Q32; q21), which juxtaposes the gene IgH with the gene Bcl2 (Antiapoptotique).
Mutations in regulatory genes such as CREBBP and KMT2D (MLL2) are very frequent and very early events and could serve as théraputiques targets.
Many other genes are found mutated, as EZH2 in 20% of the cases and various others but more rarely (sometimes in relation to the transformation).
A prognostic model including clinical and gene mutations has recently been proposed: still to be validated.
Note: Some circulating cells with T (14; 18) (Q32; q21) IGH/BCL2 are sometimes observable in the healthy subject (and are located in germ centres as non-centrofolliculaires proliferating cells). But a high number of these cells (> 1/10 000 cells) indicates a high risk of follicular NHL.
Treatment and prognosis.
Use of anti-CD20 monoclonal Ac, associated with chemotherapy.
Majority of surviving patients > 10 – 15 years.
Prognosis: Scoring system (Solal et al. Blood 2004, 104, 1258-1265).
3. Lymphoma of the coat area.
Adult lymphoma beyond 30 years, with median around 60 years, developed from cells in the lymph node coat area.
Almost constant tumor Syndrome: Adenopathies and splenomegaly, and frequent dissemination to various organs (digestive tract + +, blood in 30% of cases). Patient often from the outset at stages III or IV.
Origin.
Developed from naïve B cells
Precursor cells B with CCND1 rearrangement (but sometimes without) mature into naïve B cells that will initially colonize lymphoid organs, often in the inner part of the mantle area = This corresponds to the mantle cell tumor in situ (indolent, often accidental discovery, in combination with another lymphoma). At this stage the cells already have cytogenetic/molecular anomalies, such as the Activator mutation of ATM.
-These in situ tumors progress mainly in classical, ganglionic or extra ganglionic mantle lymphoma, usually not mutated IgVH and SOX11 +, without evidence of passage in the germ centre. Genetically unstable it acquires various additional anomalies in relation to a deregulation of the cell cycle, anomalies of the pathway of response to DNA alterations, cell survival and other pathways.
later: progression either in blastoïde mantle lymphoma (acquisition of TP53 or equivalent mutations) or in polymorphic mantle lymphoma.
-a more moderate Nb of cases comes from cells in the mantle area that undergo somatic hypermutation (presumably in the germ center), are SOX11 negative, and are genetically more stable and for a longer period than Classic coat lymphomas. They are less aggressive forms, leukemic non ganglion, which will preferentially invade the blood, the MO and often the spleen. However, they will eventually acquire additional cytogenetic/molecular anomalies, including TP53, which induce clinical and sometimes morphological progression.
CBC.
Frequent moderate anemia.
Lymphocytosis in 1/3 of cases, consisting of abnormal cells having 3 main morphological aspects:
Small cell Variant: the appearance evokes that of mature lymphocytes: The Immunophénotype directs the diagnosis
Classical: With small or medium size cells (12 – 20 Μm), with N/C ratio close to 1, whose nucleus has an irregular contour (notches, foliage appearance) and a discreetly immature chromatin
2manteau
Blastoïde form: At least part of the cells are medium or higher (15 – 25 µm), with N/C ratio close to 1, with immature chromatin with or without visible nucleolus.
Manteau2
Immunophénotype (see corresponding document).
As in the LLC-B: CD5 + and CD43 +
Unlike the LLC-B: CD23-and CD 200-(or very low)
Cytogenetics and Molecular Biology.
Quasi-constant presence of translocation t (11; 14) (q13; q32) [or one of its variants], which juxtaposes the gene IgH with the BCL1 gene (Cyclin D1: Promotes progression in the cell cycle).
Karyotypes are often complex., especially the Blastoïde variant, often associating a 17p deletion and the loss of GST p53 (very bad prognosis, low susceptibility to therapeutics).
-Transfer of ATM in 40-75% of cases.
-Mutation of TP53 (or alteration by loss of heterozygosity, translocation) and NOTCH1/2: Found in a part of the cases, are of clinical importance.
-Hyperexpression of SOX11: transcription factor expressed in the majority of NHL coat, and especially in almost all cases that do not express the Cytcline D1 (there is a monoclonal CA anti SOX11 used in Anatomo-pathology)
-Rearrangement of CCND2 (cyclin D2, most often with IgK or IgL as a partner locus): found in about half of the cases that do not show the mutation of cyclin D1 (= Diagnostic utility When cyclin D1 is negative).
Treatment and prognosis.
No cure. Polychimiothérapies, in association with an anti-CD20.
Pejorative prognosis.
Median survival < 3 years.
4. Sezary (SS) and Mycosis fungoïdes (MF) Syndrome.
These are two types of cutaneous lymphomas characterized by the proliferation of abnormal T cells in the skin: sezary cells.
MF is a localized dermatosis, of chronic evolution, of the adult and sometimes of the child.
The SS is a disease of the adult (median = 60 years), very rare, characterized by an almost generalized toxic and sometimes adenopathies.
Sezary cells are present in the skin biopsies of the 2 diseases; The diagnosis is made with cutaneous hisologie, and confirmed for the SS by the discovery of sezary cells in the blood.
The blood release is rare and limited to a few cells in the MF, whereas in the SS their number exceeds by definition 1 G/L (sometimes less in some cases), and the leukocytosis is moderate and inconsistent.
There is no cure TT in the SS, and the disease evolves over a few years. In some cases it is transformed into T-cell lymphoma with large cells.
Immunophénotype (see corresponding document)
Usually CD4 +
Sezary Cells:
Variable size, often small (that of a lymphocyte);
Cytoplasm variablely abundant and clear (no inclusions)
Core that shows deep notches or incisions, in "nail stroke" or giving a cérébriforme appearance when they are multiple.
Sez2
5. Lymphocytoses with granular lymphocytes.
They are defined by the presence during the 3 months of an excess of large granular lymphocytes (LGL or large granulate lymphocytes) > 0.5 G/L.
In the majority of cases it is a clonal expansion of T lymphocytes.
LGL
According to the WHO 2016, one identifies:
Various sub-groups and clinical pathological associations in the WHO classification 2016.
Mainly: Leukemia with large granular lymphocytes, of nature T, CD3 +, (85% of cases)
NK cell Leukemias, including chronic lymphoproliferation NK CD56 + cells (15% of cases), and aggressive NK cell leukemia, observed only in Asia.
Rare Situations, often accidental discovery, without clinical signs.
However, represent the most frequent T-lymphomas (4% of NHL).
LGL – T
Observed mainly in adults (median = 60 years).
35% of PTS are asymptomatic to diagnosis, the latter often performed during the exploration of a cytopenia discovered at CBC.
Splenomegaly in 2/3 of cases, but usually visible only to imaging.
About 60% of PTS will gradually become symptomatic with years (5-10 years): Fatigue, recurrent infections, oral ulceration (related to Cytopenias)
Auto Immune events are common (> 25% of pts)
CBC.
Anemia possible with Hb < 12 g/DL
Possible neutropenia: Moderate (0.5-1 g/L) or severe (< 0.5 g/L)
Mechanism of Cytopenias: action of LGL on myeloid precursors (release of cytokines or interaction Fas-FasL and apoptosis), and/or destruction of PNN mediated by antibodies or complex immunes
Diagnostic approach.
In a context of splenomegaly, Cytopenia (anemia, neutropenia), lymphocytosis, Auto immune disease (rheumatoid arthritis, LEAD, Hashimoto's thyroid), the discovery of a Nb of LGL > 0.5 G/L must be taken into account:
Making a blood immunophénotype (CMF)
If Nb of LGL between 0.5 and 2 G/L, a myelogram and/or a BOM with immunohistochemistry is desirable.
The search for a clonality is possible.
Immunophénotype.
These are terminal memory T lymphocytes (CD45 RA +, CD62L-), and in 90% of cases: CD3 +, CD8 +, CD57 +, CD56-, CD28-, TCR αβ + (granzyme B + en immunohistochemistry)
Evolution.
Asymptomatic Patients: Surveillance
Symptomatic Patients (anemia with Hb < 12, Moderate neutropenia with PNN > 1 g/L or severe with PNN < 0.5 G/L, Auto Immune events): Consider treatment with immunosuppressants (methotrexate or cyclophosphamide)
LGL – NK, or chronic lymphoproliferation with NK CD56 + cells
Fairly stackable to LGL-T, without racial predisposition or association with EBV, without auto immune manifestations.
Synonym of leukemia/lymphocytosis indolent with NK cells
Lymphocytes: Morphology stackable to those of LGL-T
Immunophénotype: CD2 +, CD3-, CD4-, CD8-, CD16 +, CD56 +, CD57-, KIR + [granzymes present]
Gene Configuration T: Germ
Presence of killer Ig like receptors (KIR) for diagnostic purposes (equivalent to a clonality marker)
Evolution is often less severe than that of LGL-T. If necessary treatment: It follows the same approach as that of symptomatic LGL-T.
Molecular Aspects.
A mutation of STAT3 is common in the LGL T and NK.
PTS with a STAT5B mutation form a sub-group associated with a more aggressive potential disease.
Aggressive NK cell leukemia
Disease located in Asian countries and almost always associated with EBV.
is observed more often in the young adult (median = 39 years): Brutal, rapidly gradual installation of symptoms B (especially fever), jaundice, adenopathies, hepatosplenomegaly, cytopenias and blood dissemination.
Skin infiltration is rare.
The evolution is stormy, with a median survival of 2 months (chemoresistance + +).
Abnormal cells of blood and MO have a +/-immature appearance, with nucleolus (s).
The Immunophénotype is CD2-, CD3-, CD3 ε +, CD16 +, CD56 +, CD57-
The configuration of T genes is germy.
Different chromosomal anomalies are observable.
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