O THER MALIGNANT LYMPHOPROLIFERATIVE DISORDERS The lymphomas and plasma cell problems

OTHER MALIGNANT LYMPHOPROLIFERATIVE DISORDERSThe lymphomas and plasma cell problems

MALIGNANT LYMPHOPROLIFRATIVE DISORDERS

How do you tell the difference between a neoplastic cell and an atypical lymphocyte? Neoplastic cells are “clonal” so they

all look the SAME! Atypical lymphocytes as seen in

infections will be variable. They WILL NOT all look the same.

MALIGNANT LYMPHOPROLIFRATIVE DISORDERSWhat is the difference between a

leukemia and a lymphoma?In leukemias the neoplastic cell

originates in the bone marrow and the neoplastic cells are found predominantly in the bone marrow and peripheral blood.

In lymphomas, the neoplastic cell originates in the lymph nodes or spleen and causes the development of a solid tumor.

Some lymphomas have a “leukemic phase” where the neoplastic cells are found in the peripheral blood. They may be difficult to differentiate from a leukemia.

MALIGNANT LYMPHOPROLIFRATIVE DISORDERSLymphomas

Lymphomas are divided into 2 major categories: Hodgkins lymphoma and non-Hodgkins lymphomaHodgkins lymphoma:

Can occur at any age with peak incidences at 20-30 and over 50.

Patients present to the doctor with symptoms of lymphadenopathy and may have fever, night sweats, weight loss and malaise

Diagnosis depends upon finding the Reed-Sternberg (RS) cell (something a pathologist must identify)

Are divided into 2 basic types based on the histopathology of the involved lymph nodes


Nodular lymphocyte predominance – occasional or rare RS cell seen; rare, but has a good prognosis

Classic Hodgkins lymphoma which is further subdivided into: Nodular sclerosis - bands of collagen are seen in the lymph nodes. There are mature appearing lymphocytes (T helper cells) associated with varying numbers of granulocytes, macrophages and eosinophils. Numerous RS cells are seen. This is the most common form of the disease.

Mixed cellularity – the lymph nodes contain proliferating lymphocytes, histiocytes, plasma cells, and eosinophils. There are a moderate number of RS cells Lymphocyte depleted – there are few lymphocytes

and a predominance of RS cells Lymphocyte rich – there are numerous small T

lymphocytes and occasional RS cells


Extranodal Hodgkins lymphoma – involves tumors in bone marrow, liver, or spleen

Lab features – normochromic, normocytic anemia with transitory increase in lymphocytes, monocytes, eosinophils, and sometimes platlets

Prognosis depends uponThe clinical stage of the disease (see next slide)The histiologic type of the disease – in general,

the prognosis is better the higher the number of lymphocytes and the lower the number of RS cells

TreatmentCombination chemotherapyRadiationA combination of chemotherapy and radiationCure rates are 80% or more, particularly if

diagnosis occurs in an early stage of the disease.

CLINICAL STAGING OF LYMPHOMAS


Non-Hodgkins lymphoma (malignant lymphoma)Many are associated with specific chromosomal translocations

Can be grouped morphologically by cell size intoSmallIntermediate Large

Can be grouped functionally intoB cellT cellNull cell


Can be grouped based on cell maturity intoWell differentiatedPoorly differentiated

Can be grouped based on how aggressive the disease is intoLow gradeIntermediate gradeHigh grade

LYMPHOMAS - CLASSIFICATION


Prognosis – Prognosis worsens generally with increased cell size

Prognosis worsens with decreased differentiation of the malignant cell

Low grade lymphomas have a better prognosis than high grade lymphomas

MALIGNANT LYMPHOPROLIFRATIVE DISORDERSMultiple myeloma (plasma cell

myeloma)This is usually seen in older adultsWhen the patient is first diagnosed the

following are usually seen:Multiple bone lesions with bone marrow infiltration of malignant cells

Monoclonal gamopathy (usually IgG or IgA)Generalized hypogammaglobulinemiaBence Jones proteinuria

What does this all mean?A single immunoglobulin(antibody) is produced in excess = monoclonal gamopathy


Synthesis of normal immunoglobulins is suppressed = generalized hypogammaglobulinemia

There is an overproduction of light chains resulting in light chains being found in the urine = Bence Jones proteinuria

A common complication is renal impairment

On a peripheral smear, rouleaux is the hallmark of the disease and occasional circulating plasma cells may be seen

MALIGNANT LYMPHOPROLIFRATIVE DISORDERS Waldenstrom’s macroglobulinemia

This is a plasma cell dyscrasia (abnormality) in which a monoclonal IgM is secreted.

Soft tissue involvement rather than bone marrow involvement is seen

Patients have problems with hyperviscosity of the blood.

MALIGNANT LYMPHOPROLIFRATIVE DISORDERSHeavy chain disease

This results from an overproduction of abnormal heavy chains.

Clinically the patients present with symptoms typical of malignant lymphoma.

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O THER MALIGNANT LYMPHOPROLIFERATIVE DISORDERS The lymphomas and plasma cell problems