Refers to proliferation of a single clone immunoglobulin producing cell with an increase in serum of a single homogenous immunoglobulin or its fragments.

Synonyms of PCN

nPlasma cell dyscrasia

nParaproteinemia

nMonoclonal gammopathy

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Paraprotein

nHomogenous monoclonal immunoglobulin or part of light or heavy chain

Causes of paraproteinemia

nMultiple myeloma

nWaldenstrom’s macroglobulinemia

nCLL

nNHL

nBenign monoclonal gammopathy

nHeavy chain disease

Multiple myeloma

nIt is a chronic, progressive and fatal malignant condition in which neoplastic proliferation plasma cell which infiltrate the bone marrow and other tissue.

Pathogenesis of MM

n1) Transformation of B Cell to malignant cell( Myeloma cell)

n2)Malignant cell undergo uncontrolled proliferation and infiltration.

n3) Produce similar type of immunoglobulin

n4) Secret IL- 1 and TNF, which stimulate osteoblast to secret IL-6, which stimulate osteoclast  that produce bony lesion.

n5) Monoclonal protein causes hyperviscosity syndrome.

Clinical features

nMyeloma is a disease of middle to old age, maximum incidence between 50-70 years.

nCommon- Bone pain and tenderness, anaemia, skeletal deformity and spontaneous fracture, paraplegia, seizure.

nLess common – Epistaxis,gum bleeding,infection, renal insufficiency.

Laboratory diagnosis

nBlood picture

   i) Routine -  Hb- Mild to moderate reduce

                     WBC- Commonly leucopenia   occur. May be normal or increase.

                     ESR – Markedly rise (over 100)

PBF

nRBC –Normocytic and normochromic. Marked red cell rouleaux formation present. Nucleated cells are seen.

nWBC – Immature myeloid cells are present ( LE blood picture)

nFew myeloma cells are present in 20% cases.

nPlatelet – Reduced.

Bone marrow

nCellularity : Hyper cellular, 15-30% are myeloma cells.

nGranulopoiesis, erythropoiesis and megakarryopoiesis are reduced.

Blood chemistry

n1) Total serum protein are increased due paraprotein.

n2) Serum protein electrophoresis – monoclonal M-protein present- IgG-50%, IgA-25%, IgE- 1% .

n3) Hypercalcemia

n4) Serum alkaline phosphatase and uric acid increase.

Urine examination

nBence Jones protein – are identified by boiling  and  Immunoelectrophoresis.

X-RAY

nBony change occur in 90% cases.

nChanges are either defuse decalcification or localised  destruction.

nLocalised osteolytic lesion usually appear as multiple discrete punch-out area without sclerosis. 

Myeloproliferative disorders (MPD):http://myeloproliferativedisorders.blogspot.com/

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