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Пульмонология, гематология (англ,рус)

CML. Prognosis

nThe prognosis of CML depends on a number of different parameters. Two different scoring systems are in use: one by Sokal et al (1984) and one by Hasford et al (1998).

nIn the past, median survival was 3-4 years. With interferon-based therapy, this was increased to 5-6 years.

 

Chronic lymphocytic leukemia (CLL)

nis a type of leukemia in which too many lymphocytes are produced. Although the malignant lymphocytes in CLL may look normal and mature, they are not and these cells may not cope effectively with infection.

nCLL is the most common form of leukemia in adults. Men are twice as likely to develop CLL as women. However, the key risk factor is age. Over 75% of new cases are diagnosed in patients over age 50. More than 7,000 new cases of CLL are diagnosed in the U.S. each year.

 

CLL. Essentials of diagnosis.

nMost patients asymptomatic at presentation

nSplenomegaly typical

nLymphocytosis > 5000/l

nMature appearance of lymphocytes

nCo-expression of CD19, CD5

 

Peripheral blood smear showing CLL cells

This is a microscopic view of bone marrow from a person with chronic lymphocytic leukemia; it shows predominantly small, mature lymphocytes

 

The Rai or Binet staging systems

nThese systems consider:

•The elevation of blood and marrow lymphocyte counts;

•The size and distribution of lymph nodes;

•The spleen size;

•The degree of anemia and the extent of the decrease of the blood platelet count.

 

The Rai staging system

nO stage – lymphocytosis only

nI stage - lymphocytosis +lymphadenopathy

nII stage - organomegaly

nIII stage - anemia

nIV stage - trombocytopenia

 

The Binet staging system

nA – lymphadenopathy in less than 3 areas

nB – lymphadenopathy in more than 3 areas

nC – anemia, trombocytopenia

 

CLL. Treatment

Indications for treatment are:

•falling hemoglobin or platelet count

•progression to a later stage of disease

•painful, disease-related overgrowth of lymph nodes or spleen

•lymphocyte doubling time (an indicator of lymphocyte reproduction) of fewer than 12 months

 

CLL. Treatment

nCLL treatment focuses on controlling the disease and its symptoms rather than on an outright cure. CLL is treated by chemotherapy, radiation therapy, biological therapy, or bone marrow transplantation. Symptoms are sometimes treated surgically (splenectomy removal of enlarged spleen) or by radiation therapy ("de-bulking" swollen lymph nodes).

 

oThe myeloproliferative diseases ("MPD"s) are a group of diseases of the bone marrow in which excess cells are produced. They are related to, and may evolve into, myelodysplastic syndrome and acute myeloid leukemia, although the myeloproliferative diseases on the whole have a much better prognosis than these conditions. The concept of myeloproliferative disease was first proposed in 1951 by the eminent hematologist William Dameshek. The myeloproliferative diseases are:

•Chronic myelogenous leukemia (CML)

•Polycythemia vera (PCV)

•Essential thrombocytosis (ET)

•Myelofibrosis (increased connective tissue with decreased room for production of normal blood cells).

Prepared by Orlovsky A. V., assistant, D.Ph.,

                    Murenets N. A., postgraduate

 

Methodological Instruction to Lesson № 9.

 

Multiple myeloma. Lymphomas

Hours: 5.

Working place: classroom, hospital wards.

 

Questions.

 

1. To give definition of the term “Multiple myeloma” (MM).

2. Etiology and pathogenesis of MM.

3. Clinic of MM.

4. Laboratory and instrumental diagnostic of MM.

5. Stage of MM.

6. Diagnostic criteria and MM.

7. Differential diagnostics of MM with Waldenström's Macroglobulinemia.

8. Treatment of MM.

9. The basic preparations for treatment of MM.

10. Radial therapy of MM.

 

Examples of tests

 

1. What is this multiple myeloma?

A. the lymphomas

B. the paraproteinaemias

C. the leukaemias

D. the haemolytic anaemia

2. Multiple myeloma is malignant proliferation of which cells in bone marrow?

A. Lymphocytes

B. Monocytes                                              

C. Basophiles

D. Plasmatic cells                                         

3. Name laboratory changes of Multiple myeloma asymptomatic stage.

A. increased ESR, leucocytosis, trombocytopenia

B. increased ESR, M-protein, proteinuria

C. never changes

D. leucocytosis, anaemia

4. Which symptoms will be present in case of effected bones in patients with Multiple myeloma?

A. Bone pain

B. neoplasm

C. Fracture

D. All named above

 5. What could you see on bone marrow aspirate or trephine biopsy?

A. Leucocytes infiltration

B. Plasma cell infiltration

C. T-lymphocyte infiltration

D. B- lymphocyte infiltration

 

Answers to tests:

1. B, 2. D, 3. B, 4. D, 5. B

 

References.

 

1.     Davidson’s Principles and practice of medicine (nineteenth         edition)/Christopher Haslett, Edvin R. Chilvers and others. – Edinburgh, 2002.

2.     Harrisons Principle if internal medicine (seventeenth Edition)/Fauci, Braunwald, Hasper and other. – Part 6, section 2, Chapter 105, 106.

3.     The Merck Manual of Diagnosis and Therapy (seventeenth Edition)/ Robert Berkow, Andrew J. Fletcher and others. – published by Merck Research Laboratories, 1999.

 

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