Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Acute Promyelocytic Leukemia (APL)

Treatment: acute hospital stay

The insurer denied coverage for acute hospital stay.

The denial is overturned.

According to the medical records, the patient is a male with history of diabetes, hypertension, hyperlipidemia, and transient ischemic attack (TIA). He presented to the Emergency Room (ER) with complaints of right sided weakness. He was evaluated for cerebrovascular accident (CVA) with negative magnetic resonance imaging (MRI)/ computed tomography (CT) scans. He was found to be pancytopenic and a diagnosis of acute promyelocytic leukemia (APL) was made. He was transferred to the intensive care unit (ICU) and placed on arsenic therapy. There was concern for differentiation syndrome (DS) as well as disseminated intravascular coagulation (DIC) during his early course of therapy. He was eventually downgraded to telemetry. He underwent evaluation and returned negative for disease; complete remission (CR). He was discharged home.

Among nearly 1000 patients who enrolled on prospective clinical trials that included early use of all-trans retinoic acid (ATRA; tretinoin) for APL, the incidence of early hemorrhagic death was 3.7 percent. Population-based surveys that included less highly selected APL patients indicate an early death rate of 17 to 29 percent; the increased rate of death in the population-based surveys include additional causes of death (eg, infections, differentiation syndrome, thrombotic events), but also highlight the importance of early diagnosis and prompt treatment with ATRA. (Park et al, 2011)

"Acute promyelocytic leukemia (APL) is a rare subtype of acute myeloid leukemia, which historically had been considered one of the most fatal types of this disease process. With advances in treatment regimens, namely the introduction of all-trans retinoic acid (ATRA), outcomes have drastically improved, with complete remission (CR) rates approaching 100% in ATRA-based regimens and event-free survival rates recorded as more than 90%. Furthermore, 2-year overall survival probability and disease-free survival rates exceed 90% with these regimens. Regimens have been altered in recent years with the addition of arsenic trioxide (ATO) and anthracyclines, which have further improved outcomes." (Ryan, 2018)

The patient was found to be pancytopenic and a diagnosis of APL was made. He was placed on arsenic therapy. There was concern for DS as well as DIC during his early course of therapy. APL represents a medical emergency with a high rate of early mortality, often due to hemorrhage from a characteristic coagulopathy. It is critical to start treatment with ATRA without delay as soon as the diagnosis is suspected based on cytologic criteria, and before definitive confirmation of the diagnosis has been made by genetic, cytogenetic, or immunostaining methods. Without treatment, APL has a very short median survival of less than one month resulting from uncontrolled bleeding. DS occurs in roughly one-quarter of patients with APL treated with ATRA and/or arsenic trioxide. Typical clinical findings of DS include dyspnea, fever, peripheral edema, hypotension, weight gain, pleuro-pericardial effusion, acute renal failure, musculoskeletal pain, and hyperbilirubinemia. Commonly, DS may be associated with pulmonary hemorrhage or Sweet syndrome and represents a medical emergency. Arsenic trioxide (ATO) is associated with QT prolongation electrolyte abnormalities and torsades de pointes (TdP) and requires electrocardiogram (ECG) monitoring during induction, and weekly ECG in consolidation. In this case, acute level care is required for multiple reasons for this diagnosis and was appropriate for this individual.

The health care plan did not act reasonably and with sound medical judgment and in the best interest of the patient. The inpatient hospital stay is considered medically necessary for this patient.                                          

The insurer's denial of coverage for the acute hospital stay is reversed. Medical necessity is substantiated.