[Presenter: Joan Guitart, MD, Professor of Dermatology and Pathology, Northwestern University. All treatment decisions described in this video are the opinions of Dr. Guitart. Joan Guitart, MD was compensated by Kyowa Kirin for his participation in this video.]
DR. GUITART: Blood involvement in cutaneous T-cell lymphoma can occur even at early stages of Mycosis Fungoides and, by definition, in Sézary Syndrome.1
In this video series, I will discuss hypothetical example patient cases where flow cytometry was ordered to assess blood involvement. These examples may be similar to patients you see in your practice.
Let’s discuss Patient 1 to find out why flow cytometry was ordered.
[VISUAL ON SCREEN: Image of a 70-year-old man. Not a real patient. Image courtesy of Joan Guitart, MD.]
DR. GUITART: Patient 1 is a 70-year-old male, who has been treated for plaque psoriasis for 5 years. He has patches on his legs and arms, including elbows, and lower back, and he is also experiencing pruritus—this clinical presentation is entirely consistent with psoriasis.
[VISUAL ON SCREEN: Photo of skin symptoms. Image courtesy of Joan Guitart, MD.]
DR. GUITART: Since his diagnosis 5 years ago, his treatments have included topical steroids, phototherapy, and biologics. He demonstrated a partial response to each treatment, but experienced subsequent worsening of skin symptoms. Most recently, he was treated with a TNF-alpha inhibitor, but his skin symptoms persisted.
In my experience, Mycosis Fungoides does not typically present like this. As you can see in this image, patient presented with plaques that resemble psoriasis, with thick scale and marked erythema.
[VISUAL ON SCREEN: Photo of skin symptoms. Image courtesy of Joan Guitart, MD.]
DR. GUITART: He is currently presenting with worsening skin symptoms and increased pruritus. Patches and plaques are now more extensive, currently affecting close to 10% of his body skin surface, with more pronounced erythema.
This patient is failing standard therapy for psoriasis with worsening skin signs and symptoms. So, what would be my next steps?
Because the initial presentation was clinically consistent with psoriasis, he was never biopsied. At this point—2 to 3 weeks after topical therapy was discontinued1—I would choose to do a skin biopsy. And I always keep in mind that multiple biopsies of more than one lesion having different clinical appearances may be necessary to help reach a precise diagnosis in such a typical presentations.1
If involvement of hair follicles is suspected due to alopecia, a deeper punch biopsy may be preferable.1
His workup included complete blood count, metabolic panel and serum LDH, as well as palpation of lymph nodes, and punch skin biopsies.1 Complete blood count showed a lymphocyte count of 3100 lymphocytes/μL. His serum LDH levels and metabolic panel were normal, and adenopathy was not identified.
[VISUAL ON SCREEN: Image of punch biopsy. Image courtesy of Joan Guitart, MD.]
DR. GUITART: Punch biopsy of a representative skin lesion was reviewed by a dermatopathologist with expertise in cutaneous lymphomas. At low power, we appreciate marked epithelial hyperplasia with parakeratosis in a pattern resembling psoriasis.
[VISUAL ON SCREEN: Image of punch biopsy. Image courtesy of Joan Guitart, MD.]
DR. GUITART: But at higher power, the presence of abnormal medium sized lymphocytes is noted in the intraepidermal compartment.
Immunohistochemistry analysis revealed a predominance of CD4+ T-cells with deletion of CD7 and a high CD4:CD8 ratio of 10:1. The T-cell receptor gene rearrangement assay revealed a clonal population.1
So, why was flow cytometry ordered for this patient? What clinical flags would raise suspicion of Mycosis Fungoides and lead to further investigation?
We have a patient here with lesions resembling psoriasis, but refractory to standard psoriasis treatment. His skin symptoms were worsening despite intense skin-directed and systemic therapy, with more pruritus, and more extensive skin involvement. Now we have abnormal lab results, and the pathology report raises suspicion of Mycosis Fungoides.
So for next steps, I would want to assess the implications of lymphocytosis and order flow cytometry to assess for possible blood involvement.1,2
Flow cytometry results
[VISUAL ON SCREEN: Sample report showing flow cytometry results, not from an actual patient.]
DR. GUITART: Flow cytometry results showed no detectable blood involvement. Therefore, his negative blood assessment corresponds with a B0 blood classification, along with N0 due to lack of adenopathy indicating that this patient appears to have stage 1 MF with limited skin disease without extracutaneous involvement.3
The blood flow cytometry analysis shows 3100 lymphocytes/μL with an absolute T-cell count of 2300, with the remaining 800 representing B-cells and natural killer cells.
The total CD4 count is 1600 with a normal CD4:CD8 ratio of 2.3 and small percentage of cells without CD7 or CD26 expression. This distribution of T-cells is normal indicating lack of CTCL blood involvement.3
In summary, here are some clinical considerations related to this patient’s case:
- Mycosis Fungoides can present with atypical signs
- Mycosis Fungoides can mimic psoriasis, eczema, as well as other inflammatory skin conditions, as was the case with this patient8
- While flow cytometry analysis was negative in this case, keep in mind that 1 in 5 patients with early Mycosis Fungoides may have blood involvement5
And given the rarity of the disease and the challenges with diagnosis, NCCN Guidelines recommend consultation with a specialist with CTCL expertise whenever Mycosis Fungoides or Sézary Syndrome is suspected—this was important for accurately diagnosing this patient.1
Also keep in mind recommendations from NCCN guidelines for when to consider flow cytometry:
- NCCN Guidelines recommended flow cytometry for all patients with a new diagnosis of Mycosis Fungoides with body skin surface involvement of more than 10%, and for classification T3, which is the presence of tumors1
- Based on the NCCN guidelines, I consider flow cytometry whenever Sézary Syndrome is suspected
Why should flow cytometry be considered? Because:
- It helps with accurate staging, which provides prognostic information, and may guide treatment approach2
- It also helps to establish baseline blood involvement, which can be monitored for changes over time2
When ordering flow cytometry keep in mind these considerations:
- In my experience, it is important to specify a clinical suspicion for Mycosis Fungoides or Sézary Syndrome. This will alert the pathologist to include CTCL-specific T-cell markers in the flow cytometry panel
- The recommended T-cell panel should include: CD3, CD4, CD7, CD8, CD26, and CD459
- Finally, when ordering flow cytometry, I’ve found that sending blood samples to the same laboratory may help ensure consistency in flow methodology and the report summary7
Thank you for watching, I hope this information is useful. For more information, including additional patient cases, explore www.PROBEinCTCL.com.