Recently I had a 43 years old female patient with a discrete nodulopapular rash and normal blood count and biochemistry profile. Skin biopsy suggested an acute myelomonocytic leukemia (AML). Bone marrow aspirate corresponded to a chronic myelomonocytic leukemia. Such disparate immunophenotypic profiles between the skin infiltrates and the bone marrow lesions have been already described and are not rare. Aleukemic LC predominantly occurs in less than 10% of patients with AML. It was my first case in my long dermatologic practice.
Hi
It would be helpful if you could share the results of the immunophenotyping - chronic myelomonocytic leukemia is classified as myelodysplastic syndrome (MDS) - what were the FAB classifications? Assuming MDS, finding an acute myelomonocytic (M4) phenotype in the skin suggests to me that the MDS actually might be undergoing transformation; the apparent discrepancy not really being there. It might be useful to try and do a cytogenetic analysis of the bone marrow and then see if any of the abnormalities found are present in the cells that were found in the skin.
Also, although you have surely thought of it, I am considering differential diagnosis with Sweet's syndrome (did you review the skin histopathology yourself?).
Thanks, I have read the case report and found it very thorough. It is indeed of interest to note that the bone marrow lacks clear cytogenetic abnornalities and definitely looks like myelodysplasia.
So one (very) speculative explanation for the discrepancy might be that the AML is descended from a clone that left the bone marrow or is present at numbers too low to be visible.
Regardless of the explanation, it is an interesting case that shows how dermatologists can contribute to early diagnosis and prompt treatment of hematologic malignancies.
Aleukemic leukemia cutis is a rare condition in which leukemic cells invade the skin before they appear in peripheral blood or bone marrow specimens. To describe 43 years old female patient with a discrete nodulopapular rash and normal blood count and biochemistry profile. A biopsy of these lesions confirm a dense, predominantly monocytic infiltrate of the dermis and subcutaneous tissue. Immunohistochemical stains positive for CD43 as well as monocytic markers. Bone marrow and peripheral blood examinations may not reveal leukemia. If the Bone marrow aspirate corresponded to a chronic myelomonocytic leukemia. We consider this case as Myelodysplastic syndrome (chronic myelomonocytic leukemia) in transformation to AML if the blasts increased > 20%
This case is extremely unusual in that the diagnosis must be suggest ALC and treatment with chemotherapy is indicated solely on the basis of a positive skin biopsy result.
Thank you for your remarks. I am a dermatologist and my knowledge of leukamias is very limited. Anyway, the decision how to treat the patient was taken by hematologists. I supposed it was the right one. We had a relatively young woman with a very serious diagnosis of a skin biopsy and some suspicious bone marrow changes. Can we afford to wait till the criteria for AML are fulfilled?
I saw the patient in August 2015 because of leg swelling of unknown cause, but after a compression therapy it disappeared quite quickly. After an induction and three cycles of consolidation therapy, in February 2015 she underwent unrelated bone marrow transplantation. In the period from April 2014, when a skin AML was diagnosed, to August 2015, when I saw her again, she has experienced so many health troubles! While inspecting her medical record it comes insistently to my mind: "What doesn't kill you makes you stronger."
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