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| __NOTOC__
| | #Redirect [[Chronic lymphoproliferative disorder of NK cells]] |
| {{CMG}}; {{AE}} {{AP}}
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| ==Pathophysiology==
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| ===Clinical features===
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| This pathology affects primarily the [[bone marrow]]. Generally patients are asymptomatic, although some present with diverse cytopenias, mainly [[anemia]] and [[neutropenia]]. Less frequently, patients may present with [[hepatomegaly]], [[splenomegaly]] and cutaneous lessions.
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| Chronic lymphoproliferative disorder of NK cells have been associated with other hematological tumors, [[vasculitis]], [[splenectomy]], [[neuropathy]] and [[autoimmune diseases]]. | |
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| ===Morphology===
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| Circulating NK cells tend to be intermediate size with a round nucleus and condensed chromatin, with basofilix cytoplasm and azurophilic granules. The [[bone marrow]] biopsy, instead, show intrasinusoidal and interstitial infiltration of small cells with small and irregular nucleus and pale cytoplasm<ref>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>.
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| ===Immunophenotype===
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| *Surface [[CD3]] is negative, while cytoplasmic [[CD3]] tends to be positive.
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| *[[CD16]] positive
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| *Weak expression of [[CD56]]
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| *Cytotoxic markers: [[TIA-1]], [[granzyme B]] and [[granzyme M]] positive.
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| *Diminished expression of [[CD2]], [[CD7]] and [[CD57]].
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| *Aberrant expression of [[CD5]] and [[CD8]].
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| *Diminished CD161 expression.
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| ==References==
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| {{reflist|2}}
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| {{WH}}
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| {{WS}}
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