Waldenstroms

macroglobinemia is a rare cancer of B lymphocytes.It is associated with overproduction of IgM auto antibodies. Synonyms-Lymphoplasmacytic lymphoma,Macroglobulinemia primary

 First

described by a swedish doctor JAN GOSTA WALDENSTROM in 1944 when he diagnosed a complex of hyperviscosity symptoms in patients with anemia and hepatosplenomely CLASSIFICATION: Initially considered to be related to multiple myeloma According to new WHO classificationLymphoplasmacytic lymphoma a subcategory of low grade (indolent)NHLs

Incidence:1000 to 1500 cases per year in the US.<1% of lymphomas. Age group :60 -65 years .M>F Exact etiology is unknown yet some risk factors have been identified. Association with 6p21.3 on chromosome 6 H/o autoimmune disease in the patient-pts with hepatitis,HIV infection,rickettsiosis Genetic factors:increased risk in first degree relatives. Environmental factors:exposure to pesticide,wood dust,organic solvents.

 Clonal

proliferation of terminally differentiated B-lymphocytes.

Bleeding of gums Blurred or decreased vision,dizziness Mental status changes Nose bleeds Fatigue Easy bruisability Headache Rash Vision loss Tingling of extremities-peripheral neuropathy Vision loss Weight loss Swollen glands Blanching of fingers Bone lesions extremely rare.

CBC-Low Rbc count,Low Hb ,low platelet count. Hepatomegaly,splenomegaly Increased levels of paraproteins in the blood Serum electrophoresis-M band IGM antibody levels>3gm/dl Total protein 24 hr urine protein

Blood chemistry elevated ESR,serum uric acid levels Elevated LDH values

 RBCS-Normocytic

normochromic,Polychromasia+,nucleated RBCs may be seen.Rouleaux formation not seen WBC-Reactive lymphocytes seen Platelets-decreased on smear

Marrow contains sheets of lymphocytoid plasma cells ,immature bimultinucleated plasma cells ,mature plasma cells and lymphocytes. Erythroid series is suppressed. Megakaryocytes are suppressed.

Bone marrow infiltration pattern by LPL may be of four typesNodular Diffuse Paratrabecular Interstitial

 Congestive

cardiac failure Gastrointestinal bleeding Mental changes Vision loss Peripheral neuropathy Amyloidosis cryoglobulinemia