Haematopoietic transplantation in pulmonary alveolar proteinosis associated with chronic myelogenous leukaemia

Abstract

Summary:generated by gene targeting: these mutant mice developed pulmonary disease characteristic of PAP. In these animal models haematopoietic development and function were not Pulmonary alveolar proteinosis (PAP) is a disease of unknown etiopathogenesis sometimes associated with disturbed. [4][5][6] When found in association with haematological dismalignant haematological disorders. The potential reversibility of the process in these cases seems to be orders, PAP has been considered a secondary form. 7 In a series of haematology patients with respiratory failure, the related to recovery from the underlying disease. GM-CSF has acquired an important, potentially pathogenic incidence of PAP was 5.3%. 8 It has been suggested that in such cases, PAP may be the result of a disease-related role and BMT presents one therapeutic option effective in certain forms of human PAP. We present the case of defect in the AM function 9 and/or due to treatment (chemotherapy, radiotherapy, steroids). 10 The most signifia 43-year-old female patient with Ph؉ CML. During pretransplantation evaluation, unexpected pulmonary cant complication in both primary and secondary cases is superinfection, particularly by opportunistic organisms. The infiltrates were noted in the chest X-ray, PAP being diagnosed on biopsy. In view of the progressive respirpreferred diagnostic method both in primary 11 and secondary 8 PAP is bronchoalveolar lavage (BAL). atory symptomatology and her CML being in accelerated phase, the patient underwent haematopoietic transSpontaneous remission of idiopathic primary PAP has occasionally been described. 12 The use of BAL as therapy plantation. She died on day ؉12 from invasive pulmonary aspergillosis before a response could be has improved the prognosis considerably. However, the potential reversibility of the process in secondary PAP is observed. Pathogenic implications in PAP and the role of haematopoietic transplantation in this disease are related to prior recovery from the haematological condition. 8 The possible part played by a deficiency of GMdiscussed. Keywords: pulmonary alveolar proteinosis; CML; PBSC CSF in the pathogenesis of human PAP opens up new therapeutic possibilities, such as BMT 13 or treatment with transplantation; aspergillosis GM-CSF. 14 We present a case of PAP diagnosed in a female with CML. The patient underwent allogeneic haematopoietic Pulmonary alveolar proteinosis (PAP) is a heterogenous transplantation with the objective of achieving recovery and group of congenital and acquired diseases. It is characsubsequent control of the respiratory symptomatology. terized by deposition of extracellular granular material consisting of a mixture of phospholipids and surfactant proteins within the alveoli and airways. In humans, a fatal form of Case report congenital PAP arises from a mutation resulting in surfactant protein-B (SP-B) deficiency. 1 Acquired forms may be A 43-year-old female was diagnosed with CML Phϩ in found in previously healthy patients, but the pathogenic chronic phase during ho...

Successful allogeneic bone marrow transplantation for myelodysplastic syndrome complicated by severe pulmonary alveolar proteinosis

“…Of interest, one patient with AML-M5 with sPAP survived for more than 25 months after autologous peripheral HSCT [15]. An allo-transplanted patient with chronic myeloid leukemia complicated with sPAP reportedly died soon after allogeneic HSCT [16]. Regarding MDS complicated by sPAP, only 1 patient received cord blood HSCT and survived for more than 11 months [17].…”

“…Regarding MDS complicated by sPAP, only 1 patient received cord blood HSCT and survived for more than 11 months [17]. Major causes of death in these allo-transplanted patients included invasive pulmonary aspergillosis and sepsis [14,16], indicating that HSCT for patients with sPAP-associated hematologic malignancies is relatively risky because of susceptibility to infection and pulmonary dysfunction prior to HSCT.…”

Hyperfunction of neutrophils in a patient with BCR/ABL negative chronic myeloid leukemia

“…However, hyperresponsiveness of myeloid progenitor cells to G-CSF and enhanced neutrophil functions in the terminal phase, including fMLP-stimulated superoxide production and in vitro production of IL-8 might be strongly related to the pathogenesis of PAP as well as Sweet syndrome and be some clues to elucidate at least a part of BCR/ABL negative CML. 24 1998 CML (1) SS Pertusi et al 25 1996 CML (1) SS Kannan et al 26 1995 CML (1) SS Brodkin and Schwartz 27 1995 CML (1) SS Dieguez et al 28 1993 CML (1) SS Torri et al 29 1993 CML (1) SS Feliu et al 30 1992 CML (2) SS Sanchez-Yus and Palou 31 1992 CML (1) SS Gonzalez-Castro et al 32 1991 CML (1) SS Mijovic et al 33 1991 CML (1) SS Elovaara et al 34 1990 CML (1) SS Bello Lopez et al 35 1990 CML (1) SS Cohen and Kurzrock 36 1989 CML (1) SS Hatch et al 37 1989 CML (1) SS Rauh et al 38 1989 CML (1) SS Visani et al 39 1988 CML (1) SS Marcos Sanchez et al 40 1985 CML (1) SS Odeh 41 1981 CML (1) SS Chmel and Armstrong 42 1978 CML (1) SS Rodriguez-Luaces et al 43 1997 CML (1) PAP Cordonnier et al 3 1994 CML (4) PAP Ito et al 44 1994 CML (1) PAP Miyake et al 45 1992 CML (1) PAP Watanabe et al 46 1990 CML (1) PAP Magy et al 47 1985 CML (1) PAP Aymard et al 48 1984 CML (1) PAP Green et al 49 1980 CML (1) PAP Miyashita et al 50 1977 CML (1) PAP Yamamoto et al 51 1975 CML (1) PAP Nakamura et al 52 1988 Graves (1) SS CML: chronic myeloid leukemia; PAP: pulmonary alveolar proteinosis; SS: Sweet syndrome.…”

Pulmonary alveolar proteinosis following allogeneic hematopoietic cell transplantation

“…Previous reports have identified secondary PAP associated with hematologic malignancies including myelodysplastic syndrome, 9 pediatric leukemia 11 and CML. 13 Others have reported secondary PAP following autologous transplantation, 14 cord blood transplantation 15 and cord blood transplantation and in association with parainfluenza virus 3. 10 We report a case of secondary PAP following allogeneic HCT for CML.…”

“…8 Finally, secondary PAP has been reported following diverse conditions, including toxic inhalation and associated pneumoconioses, infections such as Pneumocystis jirovecii, viral infections, or Nocardia, and both hematologic malignancies and post-hematopoietic cell transplantation (HCT) for hematologic malignancies. [9][10][11][12][13][14][15] Dyspnea and cough predominate in the clinical presentation, whereas some cases may be discovered radiographically in the absence of symptoms. Radiographic imaging commonly demonstrates diffuse bilateral alveolar opacities, with a predilection for the central middle and lower lung, and high-resolution computed tomography scan (CT) demonstrates diffuse ground-glass opacity; typical radiographic findings seen in autoimmune PAP may not be seen in secondary PAP.…”