characterized by increased

proliferation of the granulocytic cell line without the loss of their capacity to differentiate. Results in increases in myeloid cells, erythroid cells and platelets in peripheral blood and marked myeloid hyperplasia in the bone marrow Originate in a single abnormal haemopoietic stem cell

Etiology - Not clear - Little evidence of genetic factors linked to the disease - Increased incidence Survivors of the atomic disasters at Nagasaki & Hiroshima Post radiation therapy

Clinical Features
Disease is biphasic, sometimes triphasic 40% asymptomatic Chronic phase Splenomegaly often massive Symptoms related to hypermetabolism
Weight loss Anorexia Lassitude Night sweats

Clinical Features
Clinical features cont Features of anaemia
Pallor, dyspnoea, tachycardia

Abnormal platelet function

Bruising, epistaxis, menorrhagia

Hyperleukocytosis
thrombosis Increased purine breakdown : gout Visual disturbances Priapism

Lab features
Peripheral blood film
Anaemia Leukocytosis (usu >25 x 109/L, freq> 100 x 109/L WBC differential shows granulocytes in all stages of maturation Basophilia thrombocytosis

Lab features
Peripheral blood film
Anaemia Leukocytosis (usu >25 x 109/L, freq> 100 x 109/L WBC differential shows granulocytes in all stages of maturation Basophilia thrombocytosis

Lab features
Bone marrow Hypercellular (reduced fat spaces) Myeloid:erythroid ratio 10:1 to 30:1 (N : 2:1) Myelocyte predominant cell, blasts less 10% Megakaryocytes increased & dysplastic Increase reticulin fibrosis in 30-40%

Lab features
Other lab features :
NAP reduced Serum B12 and transcobalamin increased Serum uric acid increased Lactate dehydrogenase increased Cytogenetic : Philadelphia chromosome

Laboratory- summary
Lab investigation to confirm diagnosis
Full blood picture Neutrophil alkaline phosphatase Bone marrow cytogenetic

Phases
Accelerated phase
Median duration is 3.5 5 yrs before evolving to more aggressive phases Clinical features
Increasing splenomegaly refractory to chemo Increasing chemotherapy requirement

Lab features
Blasts>15% in blood Blast & promyelocyte > 30% in blood Basophil 20% in blood Thrombocytopenia Cytogenetic: clonal evolution

Phases
Blastic phase
Resembles acute leukaemia Diagnosis requires > 30% blast in marrow 2/3 transform to myeloid blastic phase and 1/3 to lymphoid blastic phase Survival : 9 mos vs 3 mos (lym vs myeloid)

General Management
Discussion with family
The disease & diagnosis Prognosis Choices of treatment
Cytotoxic drug vs bone marrow transplant Side effect

CML - principles of treatment

Relieve symptoms of hyperleukocytosis, splenomegaly and thrombocytosis
Hydration Chemotherapy (bulsuphan, Hydoxyurea)

Control and prolong chronic phase (non-curative)

alpha interferon+chemotherapy imatinib mesylate chemotherapy (hydroxyurea)

CML - principles of treatment

Treatment cont Eradicate malignant clone (curative)
allogeneic transplantation alpha interferon ? imatinib mesylate/STI 571 ?(Thyrosine kinase inhibitor)

Chemotherapy
Busulphan
Alkylating agent Preferred in older pts (not candidate for transplant) Side effect :
prolonged myelosuppression Pulmonary fibrosis Skin pigmentation infertility

Chemotherapy
Hydoxyures
Fewer side effect Acts by inhibiting the enzyme ribonucleotide reductase

Haematological remissions obtain in 80% for both drugs However disease progression not altered and persistence of Ph chromosome containing clone

Chemotherapy
Recombinant human - Interferon
Prolong chronic phase and increase survival Haematogical and cytogenetic remission Side effect
Flu like symptoms Fever and chills Anorexia Depression

NURSING INTERVENTION
Frequently monitor the client for pneumonia, pharyngitis, esophagitis, perianal cellulitis, urinary tract infection, and cellulitis, which are common in leukemia and which carry significant morbidity and mortality. Monitor for fever, flushed appearance, chills, tachycardia; appearance of white patches in the mouth; redness, swelling, heat or pain in the eyes, ears, throat, skin, joints, abdomen, rectal and perineal areas; cough, changes in sputum; skin rash.

 Check results of granulocyte counts. Concentrations less than 500/mm3 put the patient at serious risk for infection. Avoid invasive procedures and trauma to skin or mucous membrane to prevent entry of microorganisms. Use the following rectal precautions to prevent infections: Avoid diarrhea and constipation, which can irritate the rectal mucosa, avoid the use of rectal thermometers, and keep perineal are clean. Care for the patient in private room with strict handwashing practice. Encourage and assist patient with personal hygiene, bathing, and oral care. Obtain cultures and administer antimicrobials promptly as directed.

 Watch for signs of minor bleeding, such as petechiae, ecchymosis, conjunctival hemorrhage, epistaxis, bleeding gums, bleeding at puncture sites, vaginal spotting, heavy menses. Be alert for signs of serious bleeding, such as headache with change in responsiveness, blurred vision, hemoptysis, hematemesis, melena, hypotension, tachycardia, dizziness. Test all urine, stool, emesis for gross and occult blood. Monitor platelet counts daily. Administer blood components as directed. Keep patient on bed rest during bleeding episodes.

 Teach signs and symptoms of infection and advise whom to notify. Encourage adequate nutrition to prevent emaciation from chemotherapy. Teach avoidance of constipation with increased fluid and fiber, and good perineal care. Teach bleeding precautions. Encourage regular dental visits to detect and treat dental infections and disease.

THANK YOU
-KYLEXX09