Acute panmylelosis with myelofibrosis
Description
Introduction
The uncommon and severe variant of acute myeloid leukaemia (AML) known as APMF is distinguished by acute panmyeloid proliferation with a rise in blasts and bone marrow fibrosis.
The criteria for AML with myelodysplasia-related changes (AML-MRC)* are not met by this individual.
Classification: AML, not else specified (NOS) is the classification given by WHO in 2008.
One must distinguish it from:
AML-M7, or acute megakaryoblastic leukaemia
Primary Myelofibrosis (PMF) and Myelodysplastic Syndrome-Refractory Anaemia with Excess Blasts II (MIRefractory Anaemia with Excess Blasts II (MDS-RAEB II) in Myelodysplastic Syndrome with Fibrosis Primary Myelofibrosis (PMF)
Myelodysplasia-Related Changes in AML (AML-MRC)
Clinical course: Frequently deadly and rapidly progressing.
While some view it as a variation of MDS, this example emphasises APMF as an AML type.
Very bad prognosis; typical survival without vigorous therapy is about nine months.
Clinical Manifestations
Details of the case: 45-year-old man.
Acute onset, low backache that progresses quickly, weakness, and exhaustion are the symptoms (one month).
Results of the examination:
No organomegaly.
No lymphadenopathy.
No purpura or petechiae.
History: There is no family or prior history of cancer.
no past use of cytotoxic drugs.
Laboratory Investigations
Blood Findings in the Periphery: Pancytopenia
TLC: 3000/mm³
40,000 platelets/mm³
Hb: 7 g/dl
RBC structure:
minimum anisopoikilocytosis, normocytic, normochromic.
100 WBCs to 8 NRBCs.
occasional cells that drop tears.
WBC disparity:
10% blasts (occasional Auer rod, myeloblasts)
PMN: 65 percent
23% of lymphocytes
2% of eosinophils
Basophils: 0%
Cytochemistry: Smear results for myeloperoxidase (MPO) are negative.
Flow Cytometry Method:
Beckman Coulter FC 500, SSC vs CD45 gating.
4.1% gated blasts (CD45+ with low SSC).
Immunophenotype:
HLA-DR (45.9%), CD4 (46%), CD15 (21.7%), CD117 (16.5%), CD34 (46%), CD13 (55%), and CD33 (66.3%) were all positive.
CD19, CD10, CD7, CD3, CD8, CD14, CD11c, cCD79a, and cytoplasmic MPO are all negative.
Interpretation:
Unavailable megakaryocytic markers (CD41, CD61), blasts CD34+, and MPO–.
Aspirate bone marrow findings: "Dry tap" (because of severe fibrosis).
Biopsy: Hypercellular marrow with proliferation of panmyeloid cells.
Megakaryocytes that are dyspeptic (small, hypolobated/non-lobated nuclei).
Immature myeloid + erythroid precursors.
Clusters and scattered blasts (no unusual localisation).
Reticulin stain via WHO for fibrosis Myelofibrosis of grade III.
Immunohistochemistry (IHC) CD34 is a positive blast.
Glycophorin A, MPO, and CD61 (megakaryocytic) are blasts negative.
Cytogenetic and Molecular Behaviour
JAK2 mutation: wild-type negative.
Cytogenetics: Nothing unusual was discovered.
4. Differentiating Between Diagnoses
AML-M7, also known as acute megakaryoblastic leukaemia, needs at least 20% blasts, at least 50% megakaryoblasts, and CD41/CD61-positive cells.
CD34 is consistently positive in APMF but only 60% in AML-M7, indicating inconsistency in this case.
Because of their form (small, hypolobated megakaryocytes), MPO negative status, and abrupt clinical history, RAEB II MDS with fibrosis were eliminated.
Primary myelofibrosis, or PMF
Clusters of atypical large megakaryocytes with "cloud/balloon-shaped" nuclei and intrasinusoidal haemopoiesis are among the characteristics that are absent.
AML with myelodysplasia-related changes (AML-MRC): blasts <20% indicate that this is not AML-MRC.
Protocol
Management
3+7 regimen for induction chemotherapy:
12 mg/m² of idarubicin
100 mg of cytarabine per m²
High-Dose Cytarabine (HIDAC) consolidation: 4.5 g BD × 3 days, repeated × 3 cycles.
Due to the unavailability of an HLA-matched donor, an autologous haematopoietic stem cell transplant is the planned intervention.
7. Reaction & Result
Remission following treatment:
Grade III fibrosis decreased to Grade I.
Less than 5% blasts.
Pancytopenia became better.
Follow-up: At eight months, the patient was asymptomatic and there had been no recurrence.
Overall, the prognosis is usually dismal, with a mean survival of around nine months.
Aggressive early management is essential for better results.
Problems Faced in APMF Rapid Onset of Pancytopenia:
The patient experienced abrupt, severe weakness and low backache due to decreased blood cell production.
High Risk of Infections: Side effects from chemotherapy exacerbate the vulnerability to infections caused by severe neutropenia.
Symptoms of anaemia include weakness, pallor, fatigue, and dyspnoea brought on by low red blood cell counts.
Bleeding Tendency: Thrombocytopenia increases the risk of haemorrhage and makes bruising easier.
Side effects of chemotherapy include myelosuppression, mucositis, nausea, and vomiting with idarubicin and cytarabine regimens.
Procedural Complications: The diagnosis was made more difficult by the dry tap that was caused by bone marrow aspiration.
Emotional and Psychological Stress: Anxiety and panic might arise after receiving a diagnosis of a are and aggressive leukaemia variety.
Stem cell transplant preparation: Patients frequently deal with the anxiety of going through a transplant work-up and the hazards that come with it.
Comprehensive Nursing Solutions for Every Issue
1. Pancytopenia, or low platelets, WBCs, and RBCs
Regular vital sign and total blood count monitoring is necessary to identify any sudden changes or instability.
When blood transfusions are recommended for severe anaemia or thrombocytopenia, give them.
Because of the possibility of bleeding and weakness, take safety precautions and fall protection measures.
2. Prevention of Infections
Maintain rigorous aseptic technique during all surgeries and intravenous medication delivery.
Keep an eye out for infection symptoms, such as fever, chills, or localised redness, and report them right once.
Inform the patient and their family about the need of hand washing, avoiding crowds, and keeping fresh flowers and plants out of the room as protection against neutropenia.
3. Handling Anaemia
Conserve patient energy: plan activities to enable rest.
As necessary, keep an eye out for symptoms of oxygen saturation and hypoxia, such as tachycardia and disorientation.
Promote a diet high in vitamins, iron, and proteins.
Prevention of Bleeding and Haemorrhage:
Check for bleeding, bruising, or petechiae on the skin and mucous membranes.
Steer clear of rectal temperatures and intramuscular injections.
Give injection sites more pressure for a longer period of time.
5. Support for Chemotherapy Side Effects
Before and after chemotherapy, provide prescription antiemetics.
To avoid or treat mucositis, practise good oral hygiene.
Keep an eye out for indications of drug toxicity (renal, hepatic, or cardiac) brought on by rigorous chemotherapy treatments.
6. Mental Health Services
Encourage enquiries, give thorough explanations, and offer emotional support.
As needed, assist them in accessing counselling or support groups.
Include the family in updates and care planning.
7. Preparing Stem Cell Transplants
Inform patients about the procedure, potential side effects, and required pre-transplant testing.
Assist the patient during any potential isolation while undergoing transplantation.
After transplantation, keep an eye out for symptoms of graft-versus-host disease.
Prevention of Bleeding and Haemorrhage:
Check for bleeding, bruising, or petechiae on the skin and mucous membranes.
Steer clear of rectal temperatures and intramuscular injections.
Give injection sites more pressure for a longer period of time.
5. Support for Chemotherapy Side Effects
Before and after chemotherapy, provide prescription antiemetics.
To avoid or treat mucositis, practise good oral hygiene.
Keep an eye out for indications of drug toxicity (renal, hepatic, or cardiac) brought on by rigorous chemotherapy treatments.
6. Mental Health Services
Encourage enquiries, give thorough explanations, and offer emotional support.
As needed, assist them in accessing counselling or support groups.
Include the family in updates and care planning.
7. Preparing Stem Cell Transplants
Inform patients about the procedure, potential side effects, and required pre-transplant testing.
Assist the patient during any potential isolation while undergoing transplantation.
After transplantation, keep an eye out for symptoms of graft-versus-host disease.
Prevention of Bleeding and Haemorrhage:
Check for bleeding, bruising, or petechiae on the skin and mucous membranes.
Steer clear of rectal temperatures and intramuscular injections.
Give injection sites more pressure for a longer period of time.
5. Support for Chemotherapy Side Effects
Before and after chemotherapy, provide prescription antiemetics.
To avoid or treat mucositis, practise good oral hygiene.
Keep an eye out for indications of drug toxicity (renal, hepatic, or cardiac) brought on by rigorous chemotherapy treatments.
6. Mental Health Services
Encourage enquiries, give thorough explanations, and offer emotional support.
As needed, assist them in accessing counselling or support groups.
Include the family in updates and care planning.
7. Preparing Stem Cell Transplants
Inform patients about the procedure, potential side effects, and required pre-transplant testing.
Assist the patient during any potential isolation while undergoing transplantation.
After transplantation, keep an eye out for symptoms of graft-versus-host disease.
Prevention of Bleeding and Haemorrhage:
Check for bleeding, bruising, or petechiae on the skin and mucous membranes.
Steer clear of rectal temperatures and intramuscular injections.
Give injection sites more pressure for a longer period of time.
5. Support for Chemotherapy Side Effects
Before and after chemotherapy, provide prescription antiemetics.
To avoid or treat mucositis, practise good oral hygiene.
Keep an eye out for indications of drug toxicity (renal, hepatic, or cardiac) brought on by rigorous chemotherapy treatments.
6. Mental Health Services
Encourage enquiries, give thorough explanations, and offer emotional support.
As needed, assist them in accessing counselling or support groups.
Include the family in updates and care planning.
7. Preparing Stem Cell Transplants
Inform patients about the procedure, potential side effects, and required pre-transplant testing.
Assist the patient during any potential isolation while undergoing transplantation.
After transplantation, keep an eye out for symptoms of graft-versus-host disease.
Prevention of Bleeding and Haemorrhage:
Check for bleeding, bruising, or petechiae on the skin and mucous membranes.
Steer clear of rectal temperatures and intramuscular injections.
Give injection sites more pressure for a longer period of time.
5. Support for Chemotherapy Side Effects
Before and after chemotherapy, provide prescription antiemetics.
To avoid or treat mucositis, practise good oral hygiene.
Keep an eye out for indications of drug toxicity (renal, hepatic, or cardiac) brought on by rigorous chemotherapy treatments.
6. Mental Health Services
Encourage enquiries, give thorough explanations, and offer emotional support.
As needed, assist them in accessing counselling or support groups.
Include the family in updates and care planning.
7. Preparing Stem Cell Transplants
Inform patients about the procedure, potential side effects, and required pre-transplant testing.
Assist the patient during any potential isolation while undergoing transplantation.
After transplantation, keep an eye out for symptoms of graft-versus-host disease.
Preventing Haemorrhage and Bleeding:
Examine the skin and mucous membranes for petechiae, bleeding, or bruises.
Avoid intramuscular injections and rectal temperatures.
Increase the pressure at injection sites for an extended length of time.
5. Assistance for Side Effects of Chemotherapy
Prescription antiemetics should be given both before and after chemotherapy.
Maintain proper oral hygiene to prevent or cure mucositis.
Watch for signs of renal, hepatic, or cardiac drug toxicity caused by intense chemotherapy treatments.
6. Services for Mental Health
Encourage questions, provide in-depth justifications, and provide emotional support.
Help them access support groups or counselling if necessary.
Involve the family in care planning and updating.
Preventing Haemorrhage and Bleeding:
Examine the skin and mucous membranes for petechiae, bleeding, or bruises.
Avoid intramuscular injections and rectal temperatures.
Increase the pressure at injection sites for an extended length of time.
5. Assistance for Side Effects of Chemotherapy
Prescription antiemetics should be given both before and after chemotherapy.
Maintain proper oral hygiene to prevent or cure mucositis.
Watch for signs of renal, hepatic, or cardiac drug toxicity caused by intense chemotherapy treatments.
6. Services for Mental Health
Encourage questions, provide in-depth justifications, and provide emotional support.
Help them access support groups or counselling if necessary.
Involve the family in care planning and updating.
Preparation of Stem Cell Transplants:
Inform patients about the procedure, potential side effects, and required pre-transplant testing.
Assist the patient during any potential isolation while undergoing transplantation.
After transplantation, keep an eye out for symptoms of graft-versus-host disease.
8. Difficult Diagnostic Techniques
During unpleasant procedures, such as dry-tap bone marrow biopsies, reassure and assist the patient.
As required, treat discomfort during or following diagnostic procedures.
Notes
For more details visit : 10.4084/MJHID.2013.042
