Disease of Waldenström

The disease of Waldenström is a malignant Hémopathie rare, characterized by a proliferation lymphocytary monoclonal B with infiltration of the peripheral lymphoid Organes and the osseous Moelle by a monoclonal population lymphoplasmocytaire secreting a monoclonal Immunoglobuline M. It is of unknown etiology but a family genetic predisposition seems established. Three quarters of the patients are men (Sex ratio of 3 per 1). The age of predilection is between 50 and 70 years.

Clinical picture

The disease of Waldenström is generally asymptomatic. It is fortuitously discovered in front of a Sedimentation test (VS) very high since higher than 70 even 100 mm/h. One finds sometimes the following clinical signs:

progressive Asthenia;
Adenopathy S in 20 to 30% of the cases, farms, painless, nonadherent and generally cervical, axillaires or inguino-cnemial;
Splénomégalie or Hépatosplénomégalie moderate;
Syndrome hemorrhagic cutaneous, mucous even retinal;
Syndrome of hyperviscosity;
the peripheral in 5 to 10% of case, symmetrical, sensitive Neuropathy.

Biology

The Numération formulates blood is often normal. Sometimes certain nonspecific anomalies are found:

moderate arégénérative Anemia with severe, normocytaire or wrongfully macrocytaire, red blood corpuscles in rollers;
moderate Hyperleucocytosis (< 15.000) or moderated neutropenia, monocytose moderate, lyphocytose normal or moderate increase;
normal Plates or thrombopénie in 10% of the cases;
Lengthening of the time of bleeding by thrombopathy;
VS (> 70 mm to see 100 mm per 1st hour);
Hyperprotidémie > 80 to see 120 g/l;
the electrophoresis of proteins highlights a monoclonal peak béta or gamma;
the immunoelectrophoresis highlight IgM monoclonal K in 75% of the cases or L in 25% of the cases, with IgG and IgA normals or lowered;
Calcémie normal;
Protéinurie of Bence-Jones: free light chains in the urines;
weak Protéinurie total.

Myélogramme

It highlights one: a polymorphic lymphoid infiltration representing from 20 to 100% of the medullary cells

polymorphic Infiltration lympho-plasmocytaire of marrow;
Fibrosis and mastocytose reactional, inconstant;

Differential diagnosis

the myélome with IgM (2% of the myélomes): impaired renal function, protéinurie, hypercalcemy more frequent, faster evolution;
the Syndrome of Schnitzler: chronic urticaria, monoclonal osteocondensation and IgM;
rare chronic lymphoid leukemias with peak of IgM < 5 g/L;
benign monoclonal gammapathies or " of nature indéterminée" (monoclonal MGUS gammapathy off undetermined signifiance ) in IgM: the rate is often weaker, not medullary infiltration, stability over 3 years;
lymphomas not secreting;
Of IgM monoclonal can be found during infections, of carcinomes, autoimmune diseases, secreting lymphomas B.

Complications

peripheral Neuropathies
Amyloses
Hyperviscosité +++
Hemorrhages
Infections
standard Cryoglobulinémies 1: Syndrome of Raynaud, Arthralgie, Vascularity
AHAI
Thrombopathie

Treatments

The spontaneous evolution is variable and in certain cases the disease remains stable during years (2 to 10 years).

In the asymptomatic forms with a stable rate of IgM, one only proposes the therapeutic absention with monitoring.
In the symptomatic forms and/or with an increase in the rates of IgM, one proposes various types of chemotherapy, according to the tumoral stage:

In the forms slow, one proposes a mono continuous oral chemotherapy;
In the severe rapid and/or complicated forms, one proposes a polychimiothérapie;
the old subject is treated by monochimiothérapie;
In the event of demonstration autoimmune one associates the prednisone with the chlorambucil, the cyclophosphamide and the melphalan;

symptomatic Treatment of the complications and in particular of anemia;

In the event of syndrome of hyperviscosity or neuropathy, one proposes plasmaphérèses.

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