cml learning programme for nurses & other allied health care professionals ebmt nurses group

The European Group for Blood and Marrow Transplantation

CML Learning Programmefor nurses & other allied health care professionals

EBMT Nurses Group



Three different CML case studies

• Transplant as therapeutic option

• CML chronic phase

• Palliative CML care


Transplant as a Therapeutic Option

Courtesy of Erik Aerts, University Hospital Zürich, Zürich, Switzerland January 2012


Clinical Case

Clinical History:• 39 year old male • Age at original diagnosis : 39 years

Laboratory:Hb 8.3 mmol/lLeucocytes 32.0 - 10 /l⁹Thrombocytes 247 10 /l⁹Microscopic: 1% myeloblasts, 1% promyelocytes,

1% myelocytes, 8 % metamyelocytes, 21 % bars, 51% segmented nuclei, 3% basophils, 1% eosinophils, 3% lymphocytes and 4 % monocytes


The Patient’s Journey

• Diagnosis

• Allogenic Stem Cell Transplantation

• Post Stem Cell Transplantation


Clinical Case

Lifestyle considerations: - Mr. B. is 39 years old and lives alone - Truck driver - Mr. B. likes listening to country music, dancing and taking part in club activities

June 2010: diagnosed with CML Translocation t (9:22) BCR/ABL positive


Clinical Case

Mr. B.’s profile:

• Bone marrow: Cellularity: ++

Treatment: vincristine, dexamethasone and dasatinib


Clinical Case: Treatment Selection

• Treatment selection:

– Imatinib 400 mg /d from the diagnosis onwards

After 6 months therapy started to have effect


Clinical Case

Situation in May 2011:

Major molecular lymphatic blast crisis:-Leucocytes 8.5 g/l (32% Blasts)-Hb 12.2 g/dl-Thrombocytes 13 G/l-BCR-ABL: 5.08

Morphology: packed with high lymphoblast infiltration

Treatment: vincristine, dexamethasone and dasatinib


• Generally speaking, about 25% of the patients find an HLA-matched sibling donor

• Unrelated HLA-matched donor: Worldwide registry > 8 Mio HLA-typed volunteers Probability to find a matched donor 40-60% For ethnic minorities under 10%

After it was found that Mr. B. did not have a sibling donor, the databank was searched for an HLA-identical donor.

It soon became clear that there was only a small chance that a donor would be found for Mr. B.

Donor Search


Decision-making Process

Mr. B. and his family chose for the curative treatment option (Such decisions depend on age, co-morbidity, patient preferences and QoL indications) An HLA-identical donor was found

The remissions status of the patient was CR at that time


Conditioning Programme

On the 8th September, 2011, Mr. B. was admitted to the unit

We started with conditioning cyclophosphamide, ATG and total body irradiation


Conditioning for HSCT

On day 7 Mr. B. suffered from nausea and vomiting which we treated with several antiemetic drugs

Mr. B. tolerated the first dose of ATG well. The pre-medication was prednisone and Tavegyl (anti histaminicum)

In the evening, after the second dose of ATG, the patient got a fever of 38.5 ºC. After 1 g paracetamol Mr. B.’s temperature went down to 37.2 ºC


Conditioning for HSCT

•Day 3: first of 6 TBI treatments

•TBI with 6x2.2 =13.2 Gy (Lungs max 12GY)

•TBI and side effects


Haematopoietic Stem Cell Transplantation

• After the 6th TBI treatment, the patient received the stem cells from his donor

• HSCT, unrelated donor, ABO-identical

• The transplantation was completed without any complications


Haematopoietic Stem Cell Transplantation

• Haematological side-effects, like mucositis and nausea, were appearing

• Side-effects, combined with feelings of fear and impatience,

led to the patient becoming increasingly depressed

The most significant social support for the patient was his family


Post Haematopoietic Stem Cell Transplantation

•13 days after the transplant Mr. B. still suffered from oral mucositis grade 3

•Herpetic-Stomatitis (HSV-1)

•Thrombocytopenia


Post Haematopoietic Stem Cell Transplantation

• On October 16th Mr. B. went home for a couple of days

• In the afternoon Mr. B. called the ward because he had a fever (38.5 ºC)

• He then returned to the ward

• Mr. B. received antibiotics and prednisone


Discharge Day

On the 18th October the patient was discharged!


CML chronic phase

Courtesy of Thorunn Saevarsdottir, Landspitali University Hospital, Reykjavik, IcelandJanuary 2012


Clinical CaseCML Chronic phase

• 70 year old male, diagnosed with CML December 2008 (67 years at diagnosis)

• Background information: Married with 3 grown up children and 5 grandchildren; works as hospital security guard

• Presentation: Ongoing fatigue which prompted him to first consult GP. Philadelphia/BCR-ABL positive in all cells. Presented with leucocytosis, white blood cells 64 x 10E9/L, and splenomegaly

• Performance status: WHO: 0; Karnofsky: 90% (where 100 is perfect health, 0 death)


Co morbidities

• Diagnosed and operated for colon cancer 10 years earlier and treated with radiation therapy

Permanent colostomy Colon cancer in complete remission 2011

• Hypertensive, on medication: darazid and amlodipine

Blood pressure 120/80 mm Hg at diagnosis


Peripheral Blood Measurement at Diagnosis

• Total White blood cell: 64.0 x 10E9/L, immature white blood cells prominent

• Haemoglobin: 144 g/litre • Thrombocytes: 292 x109/L • Alkaline Phosphatase (ALP): 140 U/L • Gamma-glutamyl transpeptidase (Gamma GT): 238 U/L • Aspirate aminotransferase (ASAT): 53 U/L • Alanine transaminase (ALAT): 84 U/L • Lactate dehydrogenase (LD):1499 U/L • Creatinine : 115 µmol/L• Urea: 414 µmol/L• Carcinoembryonic antigen (CEA): 0.8 µg/L


Bone marrow results

• Prominent hyperplasia, Philadelphia / BCR-ABL

positive in all cells

• t9;22 translocation in 92-97% of cells

• Result: CML Chronic phase


Treatment

• Started on imatinib 400 mg daily January 9th 2009

• Experienced a little headache, but no gastrointestinal symptoms, jaundice, or musculoskeletal symptoms

Responded well to treatment: • January 28th 2009: white blood cells were 6.9 x 10E9/L• February 2009: felt better in general, less fatigue• March 2009: returned to work, strength and stamina still

recovering• June 2009: Bone marrow after 6 months imatinib showed CML in

complete remission

Blood values normal


Blood measurements after treatment

• 3 weeks on imatinib: Complete haematologic response• 6 months on imatinib: CML/CR (complete response) with

increased reticulin in bone marrow (reticulin increase can result in dry tap)

• Blood values normal ( cytogenetic/ FISH CR )• December 2010, still on imatinib 300 mg daily• Complete haematologic response - total white blood cells

5, 7 x 10E9/L


Treatment toxicity

• Experienced eye problems (discomfort looking into bright light), and musculoskeletal pain

• Reduced dose to 300 mg imatinib daily

• Side effects decreased and has not required treatment to be revised further


Palliative CML care

Courtesy of Arno Mank, Academic Medical Centre, Amsterdam, The Netherlands

January 2012


Clinical Case

• 58 year old female (55 years old at diagnosis)

• Diagnosis: Philadelphia-positive

CML in blast crisis phase

• Patient is a secretary in National Bank

• Lives with a friend, no children


Relevant history and treatment

• Diagnosis +1 year: treatment with imatinib

• Diagnosis + 2 year: • Myeloid blasted crisis• Induction treatment with cytarabine and idarubicin• Treatment with dasatinib

• Diagnosis +3 year: allogeneic SCT with matched unrelated donor (complete remission before transplantation)


Relevant history and treatment

Diagnosis +1 year +2 year +3 year Current situation

imatinib •Blastic crisis

•Induction treatment

•Dasatinib

•Complete remission

•Allogeneic SCT with matched unrelated donor


Situation last year

• Relapsed CML blastic phase • Treatment with Donor Lymphocyte Infusion (DLI), failed• GvHD colon and reactivation CML treated with valaciclovir• Respiration insufficient on basis of RSV-infection• Nutrition through PEG due to poor nutrition status

and weight loss• Vomiting & diarrhoea


Situation at admission

• Patient experienced nausea and vomiting for 4 weeks, with weight loss of more then 5 kg from reduced appetite

• Tremendous diarrhoea - more then 5 x daily• No oral intake possible• No fever or night sweats • Red coloured skin, especially on the back• Biopsies of the colon: showed graft-versus-host disease.

Treated with prednisone 40 mg per day• Multiple swellings on forehead, with aspirate blasts visible.

Palliative irradiation


Physical examination

• Weak, cachectic

• Clear, appropriate, orientated to person, place and time

• RR 140/100 mmHg, pulse 95/min, saturation 98% no

extra oxygen, AF 18/min

• Thorax: normal

Heart: no souffles

Abdomen: Les peristalsis, changing tympani

Extremities: normal colour, no oedema


Palliative situation

• No treatment options for the CML

• The prognosis is unfavourable. Discussed with patient and partner who chose hospice care

• Just before hospice admission the patient married in the hospital

• Prednisone, calcium carbasalate, esomeprazole magnesium, loperamide and fentanyl patches will be continued, as well as enteral feeding through PEG-probe

• Because of low platelet numbers, platelet transfusions will be considered if spontaneous haemorrhages occur


Summary

• GvHD

• Relapsed CML

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