blastic plasmacytoid dendritic cell neoplasm (Formally NK Cell Leukemia/Lymphoma) Ever heard of it?

Vicki:
My understanding of your husband's cancer is that yes, it used to be known as ANKL (Aggressive Natural Killer Cell Lymphoma) but is now considered something a bit different: Now it's considered an agranular hematodermic neoplasm (HN), and your searches for information will likely produce more information if you search by that term.

It was also called blastic natural killer cell lymphoma; the reason it was changed was because it was found to derive from a plasmacytoid dendritic cell, which is a subset of the cells in our immune system.

Now, the following information on NH comes from the source cited in italics directly below this paragraph. I don't normally cite such large sections of studies like this but fear I might mislead you if I don't quote heavily:

Niakosari, Firouzeh; Monalisa Sur,. "Agranular [CD4.sup.+]/[CD56.sup.+] hematodermic neoplasm: a distinct entity described in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas." Archives of Pathology & Laboratory Medicine. College of American Pathologists. 2007.

CLINICAL FEATURES

[CD4.sup.+]/[CD56.sup.+] HN has a distinct clinical presentation of primary skin lesions in 100% of the patients, as papules, nodules, or bruiselike lesions; bone marrow involvement with or without leukemic phase; and a fatal course. The skin lesions are localized at onset and become multiple and more generalized during the course of disease. Transformation into leukemic phase occurs, if the patient survives to reach this phase.

The median survival time has been 14 months, with no difference in patients presenting with or without concurrent extracutaneous involvement.

DIFFERENTIAL DIAGNOSIS

On routine hematoxylin-eosin staining, the skin biopsy shows features of a high grade undifferentiated hematolymphoid malignancy with a differential diagnosis that includes:
1. Acute myeloid leukemia (AML)
2. Human T-cell lymphotropic virus 1 associated adult T-cell leukemia/lymphoma
3. Cutaneous NK/T-cell lymphoma

TREATMENT AND PROGNOSIS

The prognosis for HN is generally poor with a median survival of 14 months. There is no difference in survival of patients presenting with or without concurrent extracutaneous disease.

The treatment is conventional chemotherapy for AML, which generally shows a good initial response followed by quick and fatal relapse in extracutaneous sites such as bone marrow.

Failure of conventional chemotherapy implies the necessity of developing alternate therapies, such as immunomodulation or immunotherapy with IL-3 or with anti-CD123 antibody.

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That study cited the following studies:
(1.) Adachi M, Maeda K, Takekawa M, et al. High expression of CD56 (N182CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol. 1994;47:278-282.
(2.) Chaperot L, Bendriss N, Manches O, et al. Identification of a leukemic counterpart of theplasmacytoid dendritic cells. Blood. 2001;97:3210-3217.
(3.) Patrella T, Comeau MR, Maynadie M, et al. Agranular CD4+ CD56+ hematodermic neoplasm (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related toplasmacytoid monocytes. Am J Surg Pathol. 2002;26:825-862.
(4.) Patrella T, Meijer CJLM, Dalac S, et al. TCL1 and CLA expression in agranular CD4/CD56 hematodermic neoplasms (blastic NK-cell lymphomas) and leukemia cutis. Am J Clin Pathol. 2004;122:307-313.
(5.) Karube K, Ohshima K, Tsuchiya T, et al. Non-B, non-T neoplasm with lymphoblast morphology: further clarification and classification. Am J Surg Pathol. 2003;10:1366-1374.
(6.) Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphoma. Blood. 2005;105:3768-3785.
(7.) Kato N, Yasukawa K, Kimura K, et al. CD2-Cd4+CD56+ hematodermic/ hematolymphoid malignancy. J Am Acad Dermatol. 2001;44:231-238.
(8.) Petrella T, Bagot M, Willemze R, et al. Blastic NK lymphomas (agranular CD4+CD56+ hematodermic neoplasms). Am J Clin Pathol. 2005;123:662-675.
(9.) Vermi W, Facchetti F, Rosati S, et al. Nodal and extra nodal tumor forming accumulation ofplasmacytoid monocytes/interferon-producing cells associated with myeloid disorders. Am J Surg Pathol. 2004;28:585-595.
(10.) Massone C, Chott A, Metze D, et al. Subcutaneous, blastic natural killer (NK), NK/T cell, and other cytotoxic lymphomas of the skin. Am J Surg Pathol. 2004;28:719-735.
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Unfortunately, I can't find much else that relates to NH. It's possible that the Journal of Experimental & Clinical Cancer Research may be a good resource?