What Is Acute Promyelocytic Leukemia?
A bone marrow malignancy in which the myeloid line of cells( red blood cells, white blood cells and platelets forming cells) has a shortage of mature white blood cells and an excess of immature progenitor (ancestor) white blood cells, called promyelocytes.
Acute Promyelocytic Leukemia (APL) constitutes 5-15 percent of all adult leukemia in the United States. APL in Italy has an annual incidence rate (frequency of occurrence of disease) of around 0.6 per 1 million populations. Both males and females are equally affected. The age of onset of the disease is about 40 years.
Unlike other leukemias, acute promyelocytic leukemia shows a good prognosis. Following treatment, the long term survival rate is up to 90%. Besides, the rate of premature death remains high, with 29 percent of APL patients dying within about 30 days of their diagnosis, among these cases of early deaths 35% of patients never received specific therapy for acute promyelocytic Leukemia. Hemorrhage (bleeding), differentiation syndrome (DS), and infection are the main causes of premature death with APL.
Sign and Symptoms
The patient may present with the following sign and symptoms:
- Symptoms associated with anemia like weakness, fatigue, and Shortness of breath (difficulty in breathing) while exertion.
- Symptoms due to decreased blood platelet count like disseminated intravascular coagulation (DIC), bleeding, or easy bruising.
- Symptoms of fever and infection due to decreased white blood cell count especially neutrophils.
Diagnosis and Treatment
Following Laboratory investigations, procedures, and genetics studies are required to make an appropriate diagnosis:
- Complete blood count with peripheral blood smears
- Liver function tests(LFTs)
- Renal Functional Tests(RFTs)
- Blood electrolyte levels
- Coagulation profile: Prothrombin time (PT) and activated partial thromboplastin time
- Fibrinogen (Factor 1) assay
- Lumbar Puncture in high-risk patients who have high WBC count before the provision of intrathecal therapy.
- Flow cytometry of CSF( cerebrospinal fluid)
- Molecular genetic analysis
- Bone marrow biopsy and aspiration
Treatment options include:
- For complete recovery or cure treatment with Arsenic trioxide (Trisenox ATO) or chemotherapy is recommended.
- In case of recurrent sudden onset of acute promyelocytic leukemia (APL) with a probability of serious hemorrhagic incidents prompt intervention, and supporting therapy for all-trans-retinoic acid (ATRA) and/or arsenic trioxide (ATO) is recommended to prevent early death.
- Bone marrow transplantation
What support can we give for Acute Promyelocytic Leukemia?
Acute Promyelocytic Leukemia is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. We provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Acute Promyelocytic Leukemia, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
YOU CAN HELP US WITH YOUR DONATION:
Without a Ribbon is a charity that works hard to aid those who suffer from rare cancers. You can help our cause in a variety of ways:
Donations – Without a Ribbon is grateful for every donation received. Giving to Without a Ribbon helps us to provide ongoing support, organise the annual gathering and subsidise the costs of our Warriors attending these conferences.
Sponsorship – If you wish to sponsor our charity, please contact us using this form.
Volunteering – We are always looking for volunteers to help with different aspects of running our charity. If you are looking for volunteer work, please feel free to contact us.