{'fraction_non_alphanumeric': 0.04449789536981359, 'fraction_numerical': 0.025856885147324114, 'mean_word_length': 4.679180887372014, 'pattern_counts': {'":': 0, '<': 1, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
The evolving management of a rare lymphoproliferative disorder–T‐cell prolymphocytic leukemia

A 62-year-old woman presented with a 6-week history of facial swelling, periorbital oedema, generalized itch, and a thickening of the skin over her neck, chest, and abdomen. She did not report any systemic symptoms. She had no significant past medical history and took no regular medications. On examination, she had marked facial oedema and erythema most marked in the periorbital area (Fig. 1) with thickened and fissured skin over upper chest, forearms, and abdomen. Full blood count showed Hb 130g/L, WBC 64.4 x 10 9 /L, and Platelets 300 x 10 9 /L. Serum LDH was 1487u/L (NR < 240). Blood film (Fig. 2, top left) showed a population of small lymphoid cells with high nuclear/ cytoplasmic ratio, infrequent nucleoli, and prominent cytoplasmic blebs. Bone marrow trephine was diffusely infiltrated with similar cells (Fig. 2, top right). Flow cytometry showed these cells to have a mature phenotype with pan T positivity (CD2, CD3, CD5 and CD7 +ve, TdT and DR ―ve). The cells were CD4 positive, CD8 negative (Fig. 3). Skin biopsy of the affected areas showed a dense T-cell infiltrate expressing CD3 and CD57 at the dermoepidermal junction but without epidermotropism (Fig. 2, bottom left and right, respectively). A T-cell gene receptor rearrangement was identified in the neoplastic population. Standard cytogenetics were normal but FISH studies identified an 11q22 deletion at the ATM gene locus. CT of chest and abdomen showed mild splenomegaly and diffuse low volume lymphadenopathy. The diagnosis was small cell variant T-cell prolymphocytic leukemia (T-PLL).