Blastic NK cell lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Agranular CD4+CD56+ hematodermic neoplasm; CD4+CD56+ hematodermic neoplasm; HDT; Blastic plasmacytoid dendritic cell neoplasm; BPDCN;

Overview

Pathophysiology

Blastic NK cell lymphoma is a type of lymphoma. It does not appear to be associated with Epstein Barr virus.[1]

Historical Perspective

  • Blastic NK cell lymphoma was first discovered by Adachi, an American hematologist, in 1994 following an unusual presentation of cutaneous lymphoma that express CD4 and CD56 antigens but no other T cell and B cell antigens.
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The deletion in 5q has been associated with the development of blastic NK cell lymphoma.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

  • [Disease name] may be caused by either [cause1], [cause2], or [cause3].
  • [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
  • There are no established causes for [disease name].

Differentiating [disease name] from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of blastic NK cell lymphoma is unknown as it is an extremely rare disorder.[2]

Age

  • Blastic NK cell lymphoma is more commonly observed amongmiddle-aged or elderly patients.

Gender

  • Males are more commonly affected with blastic NK cell lymphoma than female.

Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with Blastic NK cell lymphoma have an aggressive clinical course.
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally poor, and the patient survives almost 1 year after the diagnosis.

Diagnosis

Cells are positive for CD4 and CD56.[3][4]

Symptoms

  • Symptoms of blastic NK cell lymphoma may include the following:
  • Nodules, plaques and patches of variable sizes on skin

Physical Examination

  • Physical examination may be remarkable for:
  • Nodules, plaques and patches of variable sizes on skin

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • [Disease name] may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for blastic NK cell lymphoma is chemotherapy with CHOP or COP-like regimens.
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].

References

  1. Chan JK, Sin VC, Wong KF; et al. (1997). "Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm". Blood. 89 (12): 4501–13. PMID 9192774. Unknown parameter |month= ignored (help)
  2. Saeed H, Awasthi M, Al-Qaisi A, Massarweh S (2014). "Blastic plasmacytoid dendritic cell neoplasm with extensive cutaneous and central nervous system involvement". Rare Tumors. 6 (4): 5474. doi:10.4081/rt.2014.5474. PMC 4274438. PMID 25568744.
  3. Ng AP, Lade S, Rutherford T, McCormack C, Prince HM, Westerman DA (2006). "Primary cutaneous CD4+/CD56+ hematodermic neoplasm (blastic NK-cell lymphoma): a report of five cases". Haematologica. 91 (1): 143–4. PMID 16434387. Unknown parameter |month= ignored (help)
  4. Kim Y, Kang MS, Kim CW, Sung R, Ko YH (2005). "CD4+CD56+ lineage negative hematopoietic neoplasm: so called blastic NK cell lymphoma". J. Korean Med. Sci. 20 (2): 319–24. PMID 15832009. Unknown parameter |month= ignored (help)

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