Case Presentation: Unmutated IGHV ATM-Deleted CLL Case Presentation

Talha Munir, MD, St. James's Hospital in Leeds, United Kingdom, highlights his treatment choices for the case of a 76-year-old patient with unmutated IGHV, ATM-deleted chronic lymphocytic leukemia (CLL).

The patient in question is a 76-year-old male who was diagnosed with chronic lymphocytic leukemia (CLL) when the patient was 70 years old. The diagnosis was primarily made when the patient attended for a pre-op assessment for a prostate-related issue, and was found to have a white cell count of 16 [x 10^9] per liter. And the differential count showed that the patient's neutrophil count was 2.7 [x 10^9] per liter with a lymphocyte count of a 14 [x 10^9] per liter. The hemoglobin of the patient was normal at that point, at 156 grams per liter with a platelet count of 227 [x 10^9] per liter.

The patient then had assessments done including a flow cytometric analysis, which confirmed that these lymphocytes were abnormal lymphocytes, with a typical CLL phenotype. And the absolute CLL count at that point was 11 [x 10^9] per liter. The only test that was done at that point was the IDH re-mutation status, which confirmed that the patient had unmutated IGHV, but no other genetic markers were done on the CLL phenotype.

The patient was otherwise a fit gentleman. The only other past medical history was ischemic heart disease for which the patient was taking aspirin, 75 milligrams a day, and was taking 1 blood pressure pill in the form of ramipril at 5 milligrams a day. So over a number of years, the patient was monitored in the clinic, and had a very slow progression over 6 years. But at the age of 76, the patient came to us with worsening lymphadenopathy with some night sweats and a blood picture which was showing a hemoglobin of 102 grams per liter with a white cell count now at 160 [x 10^9] liter. The CLL count now is 144 [x 10^9] per liter with a platelet count of 89 [x 10^9] per liter.

In terms of the further workup, the patient's renal function is satisfactory with the creatinine of 89 micromoles per liter and an EGFR of 76 mils per minute with normal liver function tests. In terms of the cytogenetic profile, the patient has got an ATM gene deletion, but no evidence of TP53 deletion or mutation on next generation sequencing. So this patient is now in a stage where the patient may need treatment. This patient also had a staging CT scan, which showed that patient had lymphadenopathy above and below the diaphragm, with a spleen size of 17 centimeters.

So the question now arises that this patient has got unmutated IGHV status along with presence of ATM gene deletion, but no other high risk markers to speak of. And in context of ischemic heart disease, what treatment options would you consider in this patient?