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Chronic leukemia

Dr. Sura Obay Aldewachi

you will learn in this lecture:

CLL, signs and symptoms, blood picture and diagnosis. Clinical staging CLL CML: clinical features, laboratory findings. Leukemogenesis.

Chronic lymphocytic leukemia

CLL is the most common form of leukemia found in adults in western countries, it is rare before age of 40. It accounts for almost 50% of Leukemia occurring after the age of 60 years. The clinical course is extremely variable, it may be rapidly progressive with a fatal outcome in 1-2 years because of CLL complications, or it may be static over decades.

Signs and symptoms

Enlarged lymph nodes, liver, spleen. Recurrent infections Fatigue Night sweating Abnormal bruising (late stage symptom)

Blood Picture

The outstanding feature is a marked increase in leucocytes ,often 100x10^9/l or higher ,nearly all are mature small lymphocytes . although the disease appears to originate in marrow ,pancytopenia is a late feature .however ,autoimmune hemolytic anemia & thrombocytopenia occur as early complications in about 10% of the cases . 95%of cases are of B cell & 5% of T cell ,the leukemic B cells possess surface immunoglobulins (sIg) and show light chain restriction express CD19 and CD 20 antigens.

Diagnosis

A persistent circulating lymphocyte count of >5x10^9/l. BM lymphocytosis >30%.


Clinical Staging of CLL
Rai et al (1975) Stage 0 lymphocytosis of blood and marrow only. Stage 1 lymphocytosis and enlarged lymph nodes. Stage 2 lymphocytosis and enlarged liver or spleen or both with or without enlarged nodes. Stage 3 as with 0, 1&2 but Hb <11g/dl. Stage 4 as with 0, 1, 2, or 3 but platelet count < 100x 10^9 /l.


Hb>10g/dl. Pl>100x10^9/l Fewer than 3 sites of palpable organ involvement.
Good Prognosis,>10 years
A
Hb & pl as for A but 3 or more sites of palpable organ involvement.
Intermediate
B
Hb <10g/l , pl<100x10^9/l.
Poor prognosis, <2years
C
Binet et al (1981)

chronic myelogenous leukemia

the disease can occur at any age, but mainly at 30-40 years. It can be also called chronic myeloid leukemia and chronic granulocytic leukemia. The etiology of CML is not clear but little evidence of genetic factors linked to the disease, exposure to radiation like radiotherapy of other types of cancer.

Clinical features

The disease is biphasic and some times triphasic massive splenomegaly and gross hepatomegaly. Loss of weight, anorexia, night sweating, lassitude. Signs of impaired bone marrow function (anemia and thrombocytopenia) are inconspicuous till late in the disease. Anemia is generally moderate but increasing anemia & other features of marrow failure may indicate transformation to an acute phase . 75% of patients Myeloblastic. 25% Lymphoblastic usually (C ALL).


Lab findings
Hb low (anemia mild or abscent) Platelets increased (thrombocytosis) Increase WBC count (leukocytosis) freq> 100 109/L WBC differential shows granulocytes in all stages of maturation Basophilia (important diagnostic feature)

Lab features

Bone marrowHyper cellular (reduced fat spaces)Myeloid :erythroid ratio – 10:1 to 30:1 (N : 2:1)Myelocyte predominant cell, blasts less 10%Megakaryocytes increased & dysplasticIncrease reticulin fibrosis in 30-40%

Lab features

Other lab features : Serum uric acid increased Lactate dehydrogenase increased Cytogenetic : Philadelphia chromosome

Leukaemogenesis

Philadelphia chromosome: it is reciprocal translocation of parts chromosomes 9 and 22. 90-95% of CML patients have philadelphia chromosome. Patients with ph positive have a median survival of 42 months, while those with ph negative have only a 15 months median survivals.

Thanks




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