Friday, January 7, 2011

Different NHL.(non-hodgkin lymphoma)

PLASMA CELL NEOPLASMS


(Bonemarrow aspirate)
(Eccentric nuclei and Perinuclear Halo)

MULTIPLE MYELOMA

It is the neoplasm of PLASMA CELL
Infection of marrow macrophages with HHV – 8 IL 6 release Myeloma cell proliferation
Patients presents with
1. MULTIFOCAL DESTRUCTIVE BONE LESIONS throughout the skeletal system
VERTEBRA (MC site) > Ribs > Skull > Pelvis > Femur > Clavicle > Scapula
The focal lesion begin in the medullary cavity erode the cortical bone PATHOLOGICAL FRACTURES (MC site is vertebra)
2. Bone marrow show INCREASED NUMBER OF PLASMA CELLS (>30%)
Many variants of plasma cell can be seen: Plasma blasts, FLAME CELLS (Red), MOFF CELLS (Blue)
Many inclusions are also seen in these cells: RUSSEL BODIES cytoplasmic and DUTCHER BODIES nuclear.
3. Plasma cell infiltration of various organ tissue of the body

Clinical features
1. Due to Skeletal destruction
BONE PAIN
Pathological fracture
HYPERCALCEMIA
Punched out lesion on SKULL X RAY
2. Due to excessive abnormal monoclonal Ig
MC serum monoclonal Ig is IgG (followed by IgA)
HYPERVISCOSITY. Waldenstrom macroglobulinemia – serum proteins increases – viscosity.
HYPOGAMMAGLOBULINEMIA (Decreased production of Normal Ig)
3. RENAL FAILURE
It is due to Hypercalcemia + Light chain proteinuria + Pyelonephritis
Manifested as BENCE JONES (Ig light chain) Proteinuria
MC – IgG Kappa light chain
4. Due to bone marrow depression
Anemia
Thrombocytopenia

Clinical triad of multiple myeloma
MARROW PLASMACYTOSIS (>10%)
LYTIC BONE LESIONS
SERUM / URINE Myeloma (M) protein on electrophoresis

PERIPHERAL T CELL LYMPHOMA
Most are CD 4 +ve few are CD 8.
Has a DIFFUSE pattern of lymph node involvement
ANGIOGENESIS may be seen
Carries WORST PROGNOSIS of all NHL


CUTANEOUS T CELL LYMPHOMA
Also called MYCOSIS FUNGOIDES
Neoplastic proliferation of peripheral CD 4 + T cells characterized by the involvement of SKIN
There is infiltration of EPIDERMIS & UPPER DERMIS
Disease progression is characterized by extra-cutaneous spread, most commonly to LN & BM
SEZARY SYNDROME – A variant of mycosis fungoides in which skin involvement is characteristically manifested as GENERALIZED EXFOLIATIVE ERYTHRODERMA & CIRCULATING TUMOR CELLS

ADULT T CELL LYMPHOMA
Neoplasm of CD 4 + T cells infected with HTLV-1 virus
Seen in Japan & Caribbean
Characterized by
SKIN LESIONS (papules, plaques, ulcers)
HYPERCALCEMIA
PERIPHERAL BLOOD LYMPHOCYTOSIS
Peripheral smear Abnormal CD 4 + cells (FLOWER or CLOVERLEAF CELLS)
Another manifestation of HTLV-1 is TROPICAL SPASTIC PARALYSIS because of progressive demyelination of CNS.

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