Plasma cell dyscrasias

Muhammad Asif Zeb

Lecturer Hematology
IPMS-KMU
Plasma cell neoplasms

 Plasma cell neoplasms are a diverse

group of B cells disorders in which
monoclonal immunoglobulins are secreted
in the serum or urine
Plasma cell disorders

 Monoclonal gammopathy of undetermined

significance (MGUS)
 Plasmacytoma
 Multiple myeloma
 Waldenstroms macroglobinemia
 Light chain disease
 Heavy chain disease
 Key features of plasma cell neoplasms
neoplasms features
Multiple myeloma Lytic bone lesions
“M” spike on serum/ urine electrophoresis
Rouleaux on blood smear
>20% plasma cells in bone marrow

plasmacytoma Localized mass

Monoclonal plasma cells
Monoclonal Monoclonal serum protein
gammopathy of Bence jones protein absent
undetermined
Bone marrow plasma cells <10%
significance

Waldenstroms Hyperviscosity syndrome

macroglobulinemia ‫׀‬gM monoclonal protein
Multiple myeloma
 Multiple myeloma is a neoplastic proliferation of bone
marrow plasma cells characterized by lytic bone lesions,
presence of monoclonal proteins in serum
(paraproteinaemia) and urine
 or
 Multiple myeloma is a B-cell malignancy characterized by
a monoclonal expansion and accumulation of abnormal
plasma cells in the bone marrow compartment.



 Most frequent of the plasma cell neoplasm
 98% of cases occur over the age of 40
 Peak incidence is in the 7th decade
 Male predominance
Aetiology

 The exact aetiology of the disease is

unknown
 Cytokines especially IL6 is a potent growth
factor for myeloma
 Osteolytic lesions in this disease are due
to osteoclast-activating factor (OAF)
mainly tumour necrosis factor (TNF) and
IL1 secreted by the myeloma cells
 Gains, losses and structural alterations of
different chromosomes are frequent
 The most frequent monosomy is of
chromosome 13
 Myeloma Cells

Release of IL-6

Stimulates Production of another

cytokine RANK-ligand

Promotes differentiation and

activation of Osteoclasts

Bone pains and skeletal destruction

Clinical presentation

 Bone pain (back ache)

 Pathological fractures
 Features of anaemia: lethargy, weakness
 Recurrent infections
 features of renal failure
 Abnormal bleeding tendency
Skull X-ray showing punched out lesions
Lab diagnosis

 CBC:
 TLC: Normal/decrease
 Hb Decrease
 Platelet count: Normal/decrease
 There is usually a normochromic,
normocytic or macrocytic anaemia
 Rouleaux formation are marked in most
cases
 Neutropenia and thrombocytopenia occur
in advance diseases
 ESR raised
Bone marrow
Film of bone marrow aspirate in myeloma.
The cluster of plasma cells shows abnormal
morphology.
 Plasma Cell
Mott cells
Plasma cells crowded with Russell
bodies. An obstruction blocks the
release of Golgi secretions. These cells
can be found in any case of chronic
plasmacytosis.
 Plasma Cell
Flame Cells
Large, multinucleated plasma cells seen
in Multiple myeloma. The cytoplasm
resembles a red flame.
 Bence jones proteinuria in 20% of cases
 Increased plasma cells in the bone marrow
usually greater than 20% often with abnormal
forms
 Abnormal plasma cells appear in blood film of 15
% of patients
 Abnormal forms of plasma cells with intranuclear
inclusions that contain ‫׀‬g (dutcher bodies) are
often present
 Bone lesions
Lab diagonosis

 Monoclonal proteins in serum or urine or

both
 Serum paraprotein is IgG in two third, IgA
in one third with rare IgM or IgD or mixed
cases
 Normal serum Ig levels are reduced
(immuneparesis)
Biochemical findings

 Serum calcium elevation (hypercalcaemia)

occurs in 45% of patients
 Serum ALP is normal (except following
pathological fractures)
 Serum urea and creatinine are raised in
20% of cases
 Low serum albumin occurs with advance
disease
Serum protein electrophoresis
 Serum protein
electrophoresis in
multiple myeloma
showing abnormal
paraprotein in gamma
globulin region with
reduced levels of β
globulins
 The soft tissue forms most often occur in
the upper respiratory tract, rarely
disseminate, and are cured by resection.
The presence of M protein frequently
occurs but not always and they disappear
following radiotherapy to the primary
lesion