What is retinoic acid syndrome?

The retinoic acid syndrome (RAS) is an unpredictable but frequent complication which may develop after administration of all-trans retinoic acid (ATRA) most commonly in patients with acute promyelocytic leukaemia (APL).

What are the symptoms of differentiation syndrome?

The typical signs and symptoms of the differentiation syndrome include fever, hypotension, dyspnea, weight gain of more than 5 kg, and pulmonary infiltrates in chest radiograph.

What is APL differentiation syndrome?

Differentiation syndrome (DS; originally called “retinoic acid syndrome”) is a potentially fatal complication of treatment of acute promyelocytic leukemia (APL) with all-trans retinoic acid and/or arsenic trioxide, treatment of acute myeloid leukemia (AML) with inhibitors of isocitrate dehydrogenase (IDH; eg, IDH2 …

What causes APL?

APL is caused by a chromosomal translocation (rearrangement of material) that occurs in some of the body’s cells during a person’s lifetime (a somatic mutation ). The translocation involves the fusion of two genes : the PML gene on chromosome 15 and the RARA gene on chromosome 17.

What is retinoic acid used for?

Tretinoin is a medication used to treat acne and sun-damaged skin. It can’t erase deep wrinkles, but it can help improve the appearance of surface wrinkles, fine lines, and darks spots. Tretinoin is also known as retinoic acid.

Is retinoic acid vitamin A?

Retinoic acid is a metabolite of vitamin A that mediates the vitamin’s functions.

What is the ICD 10 code for differentiation syndrome?

C92. 40 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2021 edition of ICD-10-CM C92. 40 became effective on October 1, 2020.

What is PML-Rara?

Promyelocytic leukemia/retinoic acid receptor alpha or PML-RARA refers to an abnormal fusion gene sequence. It is a specific rearrangement of genetic material from two separate chromosomes (chromosomal translocation) and is associated with a specific type of leukemia.

What is Leukostasis?

Leukostasis is a pathologic diagnosis in which white cell plugs are seen in the microvasculature. Clinically, leukostasis is typically diagnosed empirically when a patient with leukemia and hyperleukocytosis presents with respiratory or neurological distress.

Can APL be cured?

Acute promyelocytic leukemia (APL) has become a curable disease by all-trans retinoic acid (ATRA)-based induction therapy followed by two or three courses of consolidation chemotherapy. Currently around 90% of newly diagnosed patients with APL achieve complete remission (CR) and over 70% of patients are curable.

How long can you live with APL leukemia?

Currently large cohort of patients enjoys disease-free survival of 5 years or longer. However, a disease-free status is not synonymous with a life free of physical and psychological health related to the cancer and/or its treatment.

Does retinol fade dark spots?

Retinoids minimize the appearance of wrinkles, bolster skin’s thickness and elasticity, slow the breakdown of collagen (which helps keep skin firm), and lighten brown spots caused by sun exposure.

What are the treatment options for emergency atra?

EMERGENCY ATRA (All-TRansretinoic Acid/ Tretinoin) INDICATION Induction treatment for suspected or proven Acute Promyelocytic Leukaemia (APL). TREATMENT INTENT Curative. Initial treatment to reduce adverse events from coagulopathy PRE-ASSESSMENT 1.

What are the symptoms of ATRA differentiation syndrome?

Differentiation Syndrome (ATRA syndrome/ retinoic acid syndrome) This is defined by the presence of: unexplained fever, weight gain, respiratory distress, interstitial pulmonary infiltrates, and pleural or pericardial effusion, with or without hyperleukocytosis, hypotension, oedema, hepatic, renal, multi-organ failure.

When to resume atra after retinoic acid syndrome?

When symptoms and clinical condition improve, resume ATRA at 50% previous dose during the first 4 days after the disappearance of retinoic acid syndrome, amelioration of pseudotumour cerebri or when bilirubin, AST/ALT or ALP are reduced to < 4 x ULN.

What kind of cancer drug is atra used for?

ATRA is an anti-cancer (“antineoplastic” or “cytotoxic”) chemotherapy drug. ATRA is classified as a “retinoid.”. (For more detail, see “How ATRA Works” section below). ATRA is used to treat acute promyelocytic leukemia (APL, APML).