👨‍⚕Q:How does the patient usually present in CML?

🧑‍⚕A: As follows:
• May be asymptomatic (25%).
• Splenomegaly (90%, may be huge in 10%). Patient may complain of mass or discomfort, or
heaviness or pain in left hypochondrium.
• Unexplained anaemia.
• General features such as weakness, malaise, loss of weight, night sweating.
• Repeated infection.
• Bleeding (due to thrombocytopenia).
• Hepatomegaly (in 50% cases).

👨‍⚕Q:What are the clinical phases or natural history of CML?

🧑‍⚕A: 3 phases:
• Chronic phase: In this phase, disease response to treatment and is easily controlled.
• Accelerated phase: In this phase, disease control is difficult.
• Blastic crisis: In this phase, the disease transforms into acute leukaemia, either myeloid (70%)
or lymphoblastic (30%), which is relatively refractory to treatment.

👨‍⚕Q:What are the causes of death in CML?
🧑‍⚕A: As follows:
• Blastic crisis.
• Secondary infection.
• Myelofibrosis.

👨‍⚕What is primary drug class used to treat CML ?
Answered by Dr Teba
tyrosine kinase inhibitor
imatinib
inactivate
BCL- ABL

👨‍⚕Q:What is blastic crisis? How can you suspect blastic crisis clinically?

👨‍⚕Q:What is the prognosis of CML?
🧑‍⚕A: As follows:
• With imatinib therapy, complete haematological remission in up to 95% cases and 70 to 80%
of these have no detectable BCR–ABL transcript.
• Following stem cell transplantation: 70% cure in chronic phase in young patients.
• Without treatment: median survival is 3 to 4 years. Some may survive up to 10 years.
• If blastic crisis: prognosis is poor. Median survival is 6 months.
• CML may transform to myelofibrosis

👨‍⚕Q:What are the indications of bone marrow transplantation?
🧑‍⚕A:Bone marrow transplantation is indicated if:
• The disease is not well controlled.
• The disease progress after initial control.
• For those who have accelerated phase disease

👨‍⚕Q:How to treat CML?
Pretreatment
Perform prechemotherapy screening in consultation with a hematologist-oncologist
Initiate prophylaxis and management of tumor lysis syndrome (e.g., IV fluid therapy, allopurinol).
Chronic phase
TKIs: first-line and second-line treatment
Adjunctive medical treatment or hematopoietic stem cell transplantation (HSCT) may be considered if other treatments fail.
Accelerated phase
Begin treatment with TKIs.
Consider systemic chemotherapy or HSCT, if the patient is eligible.
Blast phase: Treatment is similar to that of acute leukemia.
Aggressive systemic chemotherapy in combination with a TKI
HSCT, if the patient is eligible

Chief complaints
> Weakness for … months
> Occasional fever with cough for … months
> Lumps in the neck and inguinal region for … days.
History
History of present illness: The patient states that he was alright … months back. Since then, he has
been suffering from severe generalized weakness, which interferes with his day to day activities.
He also complains of occasional fever and cough for … months. Fever is usually low grade, not associated with chills and rigor, no evening rise of temperature, does not subside with sweating.
Cough is usually dry, not associated with difficulty in breathing or hemoptysis. For the last few days, the patient noticed
painless lumps involving both sides of the neck and inguinal region, which are gradually increasing in size.
There is no history of weight loss, bleeding from any site,polyarthritis, skin rash, contact with TB patients or history of exposure.
History of past illness
Family history
Personal history
Socioeconomic history
Drug history: Write according to the statement of the patient

General Examination
✓ The patient is ill-looking and mildly anemic
✓ No jaundice, clubbing, cyanosis, koilonychia, leukonychia or edema
✓ No thyromegaly
✓ There are generalized lymphadenopathy involving cervical, axillary and inguinal lymph nodes of
both sides. The lymph nodes are of variable size and shape, largest one being 2 × 2 cm, nontender,
firm in consistency, discrete, not adherent to the underlying structure or to the overlying skin.
There is no discharging sinus
✓ Pulse: 80/min
✓ BP: 130/85 mm Hg
✓ Temperature: 98ºF
✓ Respiratory rate: 18/min
Abdomen
Inspection:
✓ No abnormality detected.
Palpation:
✓ Liver—just palpable, 1 cm from the right costal margin in the midclavicular line, margin is sharp,
surface is smooth, nontender and firm in consistency, no hepatic bruit or rub.
✓ Spleen—just palpable, 2 cm from the left costal margin in the anterior axillary line towards the
right iliac fossa.
✓ Kidneys—not ballotable.
Percussion:
✓ No evidence of ascites (no fluid thrill, no shifting dullness).
Auscultation:
✓ No abnormality detected

Provisional diagnosis—With this history and clinical findings of lymphadenopathy and hepatosplenomegaly, I have some differential diagnoses ?
👨‍⚕🤔

👨‍⚕Q. What investigations do you suggest in CLL?

🧑‍⚕
A. As follows:
1. CBC, showsL
✓ Hb% (low)
✓ Leukocytosis (50 to 200 × 109
/L)
✓ Differential count (DC) shows increased lymphocytes (95%), mostly small lymphocytes
✓ Platelet is normal, low or slightly increased.
2. Bone marrow (increased lymphocytes).
3.

Others:
Reticulocyte (high in autoimmune hemolytic anemia)
✓ Coombs’ test (positive in autoimmune hemolytic anemia)
✓ Paraproteins (may be increased)
✓ Uric acid (high)
✓ Immunophenotyping of B-cell antigen (CD19 and CD23) and T-cell antigen (CD5).

👨‍⚕ Q. What is chronic lymphatic leukemia?