Case Number : Case 2450 - 22 November 2019 Posted By: Dr. Richard Carr

pan dermal dense lymphoid infiltrate both perivascular and perifollicular

green zone is there

CD 3 few cells positive, sir can we have CD20

CD20 negative.

Mmmm... CD123? CD56? or please widen the T-cell and B-cell panel, in case this is loss of antigen expression.

Agree with Dr Salem. I’ve seen a similar case recently. Cd4+ cd56+ cd123+. Blasting dendritic plasmocytoid Lymphoma.  Is the patient treated for myeloid leukemia?

CD56 and Cd 123 staus, Blood smear and MPO stain

sir the battery of round cell tumors comes into differential diagnosis. How common is double negative lymphomas in the skin

Will check CD5,CD7 and CD79a before going to a full work up of leukemic deposits including work up for BPDCN. The duration is only 6 weeks and this will be more with a leukemic deposit or secondaries of nodal disease.  

This is a BPDCN (CD4+, CD56+) recurrence. Remarkably about 18 months down the line from chemotherapy treatment. You should be aware there are now some promising treatments e.g. anti-CD123 (FDA approved)

MD Anderson: Dr Naveen Pemmaraju (Lead Clinician)
On BPDCN cells there’s a marker called CD123. Tagraxofusp, a targeted therapy we’ve helped develop and study, attaches to that marker and then kills the cancer cells. The results are very encouraging. Over 90% of patients who received tagraxofusp as their first treatment had a major response. That means that the skin lesions and/or bone marrow lesions disappeared. A paper we recently published in the New England Journal of Medicine shows that we’re more than two years into our study, and we still haven’t reached the median length of survival for these patients. So we’ve apparently at least doubled the survival time when comparing to this historic expectation for our patients. The U.S. Food and Drug Administration approved tagraxofusp for treatment of BPDCN in December 2018; it’s now considered one of the standard treatments for this disease.

https://www.nejm.org/doi/full/10.1056/NEJMoa1815105

sir great case