Dysglobulinemic Neuropathy MECHANISM FIG 1. MECHANISM ANTI MAG NEUROPATHY FIG 1. MGUS-IGM AND POLYNEUROPATHY. DIRECT IMMUNOFLUORESCENCE(IF). SOME MYELIN SHEATHS ARE SPECIFICALLY LABELLED BY ANTIIGM-NEUROPATHY. FIG 2. INDIRECT IF WITH SERUM FROM 1 WITH ANTI-IGM ANTIBODY. ALL THE MYELIN SHEATHS OF THIS NORMAL HUMAN NERVE ARE LABELLED. FIG 3. MGUS-IGM. EM.WIDENINGS OF THE MYELIN LAMELLAE. FIG 4. MGUS-IGM. EM.WIDENINGS OF THE MYELIN LAMELLAE. FIG 5. MGUS-IGM. EM.WIDENINGS OF THE MYELIN LAMELLAE. INTRA-MYELINIC DEPOSITS FIG 1. DIRECT IMMUNOFLUORESCENCE (IF). MGUS-IGA. SEVERAL MYELINATED FIBERS ARE SPECIFICALLY LABELLED BY AN ANTI-IGA ANTIBODY. FIG 2. INDIRECT IF WITH THE SERUM OF PATIENT 1 LABELLED WITH AN ANTI-IGA ANTIBODY. ALL THE MYELINATED FIBERS OF THIS NORMAL HUMAN NERVE. FIG 3. SAME CASE AS 1. WIDENINGS OF THE MYELIN LAMELLAE.EM TRANSVERSE SECTION. FIG 4. SAME CASE AS 1. LONGITUDINAL SECTION CLOSE TO A NODE OF RANVIER. WIDENINGS OF THE MYELIN LAMELLAE. FIG 5. SAME CASE AS 1. WIDENIGS OF THE MYELIN LAMELLAE AT HIGH MAGNIFICATION. FIG 6. SAME CASE AS 1. IMMUNOEM. WIDENINGS OF THE MYELIN LAMELLAE ARE SPECIFICALLY LABELLED BY AN ANTI-IGA ANTIBODY. ENDONEURIAL DEPOSITS FIG 1. WALDENSTROM DISEASE. DIRECT IMMUNOFLUORESCENCE. ANTI-IGM ANTIBODY. NUMEROUS LARGE DEPOSITS LOCATED IN THE ENDONEURIAL SPACE ARE POSITIVE. FIG 2. SAME CASE AS 1. ABNORMAL ENDONEURIAL DEPOSITS ARE ERASING THE SCHWANN CELL BASAL LAMINAE. BARR=0,2µ. FIG 3. WALDENSTROM DISEASE. PRESENCE OF HUGE AMORPHOUS ENDONEURIAL DEPOSITS.A FEW MYELINATED FIBERS ARE SEEN. FIG 4. SAME CASE AS 3. ONE MYELINATED FIBER AND UP PRESENCE OF LARGE ENDONEURIAL DEPOSITS. BARR=1µ. FIG 5. SAME CASE AS 3. IMMUNOEM. THE ENDONEURIAL DEPOSITS ARE SPECIFICALLY LABELLED BY ANTIIGM-ANTIBODY. BARR=0.2. FIG 6. EM. SMALL IGG DEPOSITS IN THE ENDONEURIAL SPACE. THEY ARE TOO SMALL TO BE SEEN BY OPTIC MICROSCOPE. AT HIGH MAGNIFICATION (SEE 2 ). FIG 7. EM. SAME CASE AS 6. HIGH MAGNIFICATION OF A SMALL ENDONEURIAL DEPOSIT; FINGERPRINT ASPECT (CRYOGLOBULIN). BARR=0,05µ. FIG 8. SAME CASE AS 6. AT HIGH MAGNIFICATION. FINGER PRINT ASPECT. FIG 9. SAME CASE AS 6. CRYOGLOBULIN ISOLATED FROM BLOOD PATIENT. SAME STRUCTURE THAN THE DEPOSITS IN THE ENDONEURIAL SPACE. BARR=0,O5µ. FIG 10. SAME CASE AS 6. A SMALL CRYO DEPOSIT IS SEEN EN THE LUMEN OF THIS ENDONEURIAL CAPILLARY. FIG 11. SAME CASE AS 6. SMALL CRYO DEPOSITS ARE CROSSING THE WALL OF AN ENDONEURIAL CAPILLARY. FIG 12. WALDENSTROM DIDEASE. EM. SMALL GRANULAR DEPOSITS ARE SEEN IN THE ENDONEURIAL SPACE. BARR=0,3µ. FIG 13. SAME CASE AS 12. IMMUNO EM. THE SMALL ENDONEURIAL DEPOSITS ARE SPECIFICALLY LABELLED BY AN IGM ANTIBODY. BARR=0,2µ. INTRA-MYELINIC AND ENDONEURIAL DEPOSITS FIG 1. SMOLDERING MYELOMA. SEVERE RAREFACTION OF MYELINATED FIBERS. FIG 2. SAME CASE AS 1. EM. OUTFOLDINGS OF MYELIN LAMELLAE WHICH ARE OFTEN ABNORMALLY WIDENED. BARR=0.4µ. FIG 3. SAME CASE AS 1. EM. MAGNIFICATION OF THE WIDENINGS OF THE MYELIN LAMELLAE. THE INTRAPERIODIC LINES ARE INTACT. BARR=0.05µ. FIG 4. SAME CASE AS 1. EM. PRESENCE OF NUMEROUS GRANULAR GRAY DEPOSITS IN THE ENDONEURIUM AMONG COLLAGEN FIBRILS(C). BARR=1µ. FIG 5. SAME CASE AS 1. IMMUNOEM. IN THE ENDONEURIUM CLOSE TO COLLAGEN FIBRILS(C), DEPOSITS ARE SPECIFICALLY LABELL. BARR=0.1µ. FIG 6. SAME CASE AS 1. IMMUNOEM. PRESENCE OF IgG AT THE LEVEL OF THE WIDENINGS. BARR=0.1µ. FIG 7. SAME CASE AS 1. IMMUNOEM. IgG IN THE WIDENINGS OF THE MYELIN LAMELLAE AND OUTSIDE THE SCHWANN CELLS (D). BARR=2µ. FIG 8. SAME CASE AS 1. ELECTRON MICROSCOPY. ULTRASTRUCTURAL ASPECT OF THE CRYOPROTEIN ISOLATED FROM THE SERUM. BARR=0.05µ. FIG 9. SAME CASE AS 1. IMMUNOEM. SPECIFIC LABELLING BY ANTI IgG ANTIBODY OF THE CRYOPROTEIN ISOLATED FROM THE SERUM. BARR=0.1µ. CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP) FIG 1. EM. Transverse section. IGM monoclonal dysglobulinemia. Demyelinating lesions and numerous mac in the endoneurial space. CIDP. FIG 2. EM. Transverse section. Same case than fig 1. A mac is entered a Schwann cell and splits the myelin sheath. FIG 3. Transverse section. Same case than fig 4. A mac is surrounding a Shwann cell in which the myelin sheath has been destryed, the axon is naked. FIG 4. EM. Transverse section. MGUS-IGG. CIDP. A mac full of myelin debris is inside the Schwann cell; on the left note the naked axon. CHEMOTHERAPY INDUCED NEUROPATHY FIG 1. FIG 2. Paraffin embedded transverse section.Anti-KP1. Waldenstrom disease;chemotherapy induced-neuropathy. A few macrophages are located around an epineurial vessel and in the endoneurial space. FIG 3. Transverse semi-thin section. Same case fig 2. Several ovoids and a few clusters are seen;axonal neuropathy. FIG 4. EM. Transverse section. Same case fig 2. Axonal lésion; ovoide.