Pure White Cell Aplasia: Report of the First Case Associated with Primary Biliary Cirrhosis

Tamura, Hideto; Okamoto, M; Yamashita, Takenari; Sato, Chikara; Watanabe, Ayako; Kondo, Asaka; Tatsuguchi, Atsushi; Tsuji, Takashi; Ogata, Kíyoyuki; Dan, Kazuo

doi:10.1532/ijh97.06018

Cited by 10 publications

(13 citation statements)

References 17 publications

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“…4 14 It should be noted, unlike our case, that the condition is largely been seen in older patients 4 5 6 11 14 16. There has been one documented case of PBC and PWCA 16. This is to our knowledge the first documented case of AIH and PWCA.…”

Section: Discussionmentioning

confidence: 51%

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Pure white cell aplasia: report of first case associated with autoimmune hepatitis

Keast

Weeraman

Mayhead

et al. 2014

Frontline Gastroenterol

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Pure white cell aplasia (PWCA) is a rare, immune-mediated condition that causes a profound inhibition of myelopoiesis. It has been seen in association with other autoimmune conditions, thymomas, chronic lymphocytic leukaemia and as an adverse drug reaction. We report what we believe to be the first case of PWCA associated with autoimmune hepatitis. An 18-year-old woman presented with jaundice and was found to be suffering from acute hepatitis. Later she became neutropenic and lymphopenic. Liver biopsy was indicative of autoimmune hepatitis. This was later confirmed by autoantibody results. Bone marrow aspirate found an absence of all myeloid progenitor cells, highly suggestive of PWCA. The low white cell count only improved after immunosuppression with corticosteroids. Her condition remains stable 1 year later with azathioprine therapy.

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Section: Discussionmentioning

confidence: 51%

“…As in this case, it commonly follows a relapsing and remitting course6 13 – 15 and death from sepsis is frequently reported. 4 14 It should be noted, unlike our case, that the condition is largely been seen in older patients 4 5 6 11 14 16. There has been one documented case of PBC and PWCA 16.…”

Section: Discussionmentioning

confidence: 53%

Pure white cell aplasia: report of first case associated with autoimmune hepatitis

Keast

Weeraman

Mayhead

et al. 2014

Frontline Gastroenterol

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“…Clinically, patients with PWCA suffer from recurrent infections. Previously, thymoma and thymic carcinoma (5)(6)(7) as well as drug-induced (8)(9)(10)(11)(12) and autoimmune diseases (13)(14)(15) were reported in PWCA patients. The immunologic mechanisms have been demonstrated in some PWCA cases where an inhibiting antibody was found (15).…”

Section: Introductionmentioning

confidence: 99%

Pure White Cell Aplasia Complicated by Systemic Sclerosis with Accompanying Scleroderma Renal Crisis

et al. 2022

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Pure white cell aplasia (PWCA) is a rare neutropenic disorder caused by absence of neutrophil-lineage cells. A 47-year-old man was diagnosed with scleroderma renal crisis 2 months prior to admission to Ohta-Nishinouchi Hospital after experiencing a fever and abdominal pain. Blood tests revealed severe neutropenia, and bone marrow aspirate showed the absence of neutrophil-lineage cells. He was diagnosed with PWCA. Steroids alone were not effective, but adding cyclosporine A and high-dose immunoglobulin recovered his neutropenia and improved his condition. Cyclosporine A and high-dose immunoglobulin are thus considered effective for treating PWCA in autoimmune diseases.

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“…The main pathogenetic mechanism of PWCA is considered to be the immune-mediated suppression of granulopoiesis, with autoantibodies against the progenitor cells of granulocytes 1 and subsequent inhibition of granulocyte-macrophage colony-forming units 2 . PWCA is commonly associated with several immune diseases (autoimmune hepatitis and primary biliary cirrhosis 3,4 , autoimmune thyroiditis with autoantibodies to striated muscle cells and CD8 + naive cells 5 ). There have been described cases of recurrent PWCA after bone marrow transplantation, successfully treated with biological drugs [6][7][8][9] .…”

Section: Introductionmentioning

confidence: 99%

A rare case of pure white cell aplasia in a patient with thymoma complicated by infective endocarditis

Dvoretsky¹,

Yakovlev²,

Суворова³

et al. 2021

Arch Balk Med Union

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Un cas rare d'aplasie pure des globules blancs chez un patient avec un thymome compliqué d'une endocardite infectieuseIntroduction. L'aplasie pure des globules blancs (PWCA) est une maladie hématologique rare caractérisée par l'absence de lignées de neutrophiles dans la moelle osseuse avec mégacaryopoïèse et érythropoïèse intactes. La PWCA est généralement associée à plusieurs pathologies immunitaires. La cytopénie chez les patients atteints de thymome est considérée comme l'une des maladies des syndromes paranéoplasiques avec d'autres manifestations. Les deux principaux problèmes cliniques de la PWCA sont diverses complications infectieuses et les lésions ulcéreuses-nécrotiques de la peau et des muqueuses. Rapport de cas. Cet article décrit le cas rare d'un patient atteint de thymome et de l'un de ses syndromes ABSTRACT Introduction. Pure white cell aplasia (PWCA) is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow, with intact megakaryopoiesis and erythropoiesis. PWCA is commonly associated with several immune pathologies. Cytopenia in patients with thymoma is a paraneoplastic syndrome, along with other manifestations. The two major clinical issues in PWCA are various infectious complications and ulcerative-necrotic lesions of the skin and mucosa membranes. Case presentation. We present a rare case of a patient with thymoma and PWCA, as paraneoplastic syndrome. On the background of agranulocytosis, the patient developed sepsis and infective endocarditis (IE) caused by a rare agent, carbapenem-resistant Klebsiella pneumoniae.Conclusions. The rarity of endocarditis caused by Klebsiella species limits its recognition and awareness

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Pure White Cell Aplasia: Report of the First Case Associated with Primary Biliary Cirrhosis

Cited by 10 publications

References 17 publications

Pure white cell aplasia: report of first case associated with autoimmune hepatitis

Pure white cell aplasia: report of first case associated with autoimmune hepatitis

Pure White Cell Aplasia Complicated by Systemic Sclerosis with Accompanying Scleroderma Renal Crisis

A rare case of pure white cell aplasia in a patient with thymoma complicated by infective endocarditis

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