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WELCOME

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Case Presentation

Dr. Morsheda Akter Jhorna

MD Resident (Phase-A)

Yellow Unit

Department of Haematology

BSMMU

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A 55-year-old male presented with abdominal distension & generalized weakness

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Particulars of patient :

  • Name:Kohinoor
  • Age:55years
  • Sex:Male
  • Religion:ISLAM
  • Occupation:Businessman
  • Marital status:Married
  • Address :Keraniganj
  • Date of admission: 12/3/22
  • Date of examination :12/03/2022

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Presenting complaints :

  • Generalized weakness for last 4-5 months
  • Abdominal fullness & discomfort for same duration.
  • H/O feeling a swelling in left upper abdomen for same duration.

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H/O Present Illness:

According to the statement of patient he was reasonably well for 4-5 months back

Then he developed generalized weakness which was increased than before around within 4-5 months

He also felt a swelling in left upper abdomen which was increasing day by day & it creates heaviness, fullness & discomfort for same duration which was not associated with food.

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He gave H/O loss of appetite, loss of weight about 5 kg within this time which is unintentional .

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Past medical history

  • He was diagnosed as a case of CML since 2012.
  • He took Cap Hydroxyurea 500 mg,
  • Tab Imatinib 400 mg,
  • Tab Dasatinib 100 mg irregularly.
  • He completely stop his medication from 2019 to 2020.
  • Now patient admitted to hospital for further management.

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He is diabetic for last 3 months

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Family history:

Parents are alive.He has 3 siblings.

All of them are doing well.

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Personal history :

No history of betel nut chewing,smoking or taking alcohol.

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Immunization history:

He is immunized according to EPI schedule. He gave no history of adult immunization.

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On general examination :

  • Appearance: Ill looking
  • Body built: Below average
  • Co-operation: Co-operative
  • Decubitas: On choice
  • Anaemia: Mild
  • Jaundice: Absent
  • Clubbing: Absent
  • Koilonychia: Absent
  • Leuconychia: Absent
  • Edema : Absent

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  • Dehydration: Absent
  • Pigmentation: Normal
  • Lymph node: No palpable lymphnode
  • Thyroid gland: Not enlarged
  • Neck vein: Not engorged
  • Pulse: 78 b/p/m
  • Bp: 110/80 mm hg
  • Temperature : 98 F
  • Respiratory rate: 20/min

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On systemic examination :

  • Abdomen
  • On inspection :
          • Distended
          • Umbilicus is centrally placed & everted.
          • No dialated veins,scar or sinuses.
          • No visible peristalsis or any other pulsation.
          • Hernial orifices are intact.

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Palpation

  • Superficial
    1. Local temperature –Normal
    2. No tenderness
    3. No rigidity
  • Deep
    • Liver : Not palpable.
    • Spleen : There is splenomegaly measuring about 6cm from left costal margin along its long axis towards the right iliac fossa , firm in consistency , non tender , percussion note dull , there is no splenic bruit.
    • Kidneys : Not ballotable .

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Percussion

  • Upper border of the liver dullness :Right 5th intercostal space along the mid-clavicular line.
  • Splenic dullness present from mid-clavicular line towards rt iliac fossa.
  • No evidence of ascites.(No fluid thrill , no shifting dullness).

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Auscultation

  • Bowel sound present
  • No hepatic bruit.
  • No renal or aortic bruit.
  • No splenic rub

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  • Cardiovascular system
  • Respiratory system
  • Central nervous system

Reveals no abnormality

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Investigations

CBC:

Hb:9.1 gm/dl

ESR:42

RBC count:3.32 m/cumm

HCT/PCV:27.5%

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Cont.

Total WBC count: 50x10^9/L

Differential count:

Neutrophil:50%

Lymphocyte:20%

Monocyte:02%

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Eosinophil:02%

Basophil:00%

Blast: 16%

Myelocyte:10%

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Bone Marrow Examination

  • Cellularity:Grossly hypercellular
  • Myeloid/Erythroid ratio:Increased
  • Erythropoiesis:Relatively depressed
  • Granulopiesis:Grossly hyperactive and shows left shift with predominant cells are blasts(Approximately 15%) and intermediate forms though segmented forms are also present.
  • Megakaryocytes:Present but fewer than normal
  • Lymphocyte scanty
  • Plasma cell :Scanty
  • Histiocyte:Scanty
  • Ectopic cell :Not found
  • Cytochemistry:Not found

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Comments:

Strongly suggestive of Chronic myeloid leukemia in accelerated phase

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BCR/ABL1 Gene ANALYSIS

Result:Positive

BCR/ABL1 p210 mRNA were detected

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BCR/ABL1 observed copies

1021.001 copies/reaction

ABL1 observed copies

15011.475

BCR-ABL1/ABL1 transcript percent ratio:

6.80%

Conversion factor for IS:

0.6289%

BCR-ABL1/ABL1 IS[%]

4.28%

BCR-ABL(Minor) p190 t(9:22)

Not detected

BCR-ABL(Micro) p230 t (9:22) translocation

Not detected

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Cont.

Flow cytometry and TKI mutations was not done.

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  • What will be the povisional diagnosis?
      • Chronic myeloid leukemia with Accelarated phase

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  • Differential Diagnosis :
      • Myelofibrosis

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What will the further management in this patient??

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Chronic myeloid leukemia

CML is a MPN characterized by rearrangement of long arm of chromosome 9 and 22, resulting in Philadelphia chromosome , creating fusion oncogene BCR-ABL 1.

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Incidence

  • 1-2 per 100000
  • Men>women
  • Age usually after middle forties

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Etiopathogenesis

  • Evidence from epidemiological study of Hiroshima & Nagasaki atomic bomb survivors, ionizing radiation is a known risk factor

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Phases of CML :

      • Chronic phase
      • Accelerated phase
      • Blast crisis

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Treatment Options

  • Targated Therapy
  • Tyrosine Kinase Inhibitor

TKIs used to treat CML:

      • Imatinib
      • Dasatinb
      • Nilotinib
      • Ponatinib
      • Bosutinib
    • Asciminib :It may be useful either in combination with another TKI or in the event of resistance mediated by a mutation of the ABL kinase.
  • Chemotherapy:
      • Hydroxycarbamide

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Cont.

  • Alpha Interferon
  • Stem Cell Transplantation

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  • Lymphoblastic transformation approximately one-fifth of cases.
  • Treatment is similar to ALL
  • Transformation into AML or mixed type in majority cases and difficult to treat.
  • Survival rare without SCT.

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Criteria for response

  • Complete hematological response:
    1. Normalization of CBC,Leukocyte count <10000/cc
    2. Platelet count <450000/cc
    3. No immature cells in peripheral blood.
    4. No s/s of disease with resolution of palpable splenomegaly.
  • Cytogenetic response
  • Molecular response

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Follow up

  • In general patient receiving TKI therapy should have BCR-ABL1 testing every 3 month for the first 2 years.Then every 3,6,12 month if MMR is achieved.

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Thank You