Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0206061 (interstitial pneumonia)
 6,105 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

As typical disorders of the elderly, myelodysplastic syndromes (MDSs) are relatively unusual in childhood. Nevertheless, up to 17% of cases of pediatric acute myeloid leukemia may have a preleukemic phase. In young patients, the goal of treatment is eradication of the preleukemic malignant clone and reconstitution of normal hematopoiesis. Allogeneic bone marrow transplantation (BMT) has proved to be capable of this, but the optimal conditioning treatment to achieve eradication remains to be defined. Between May 1989 and June 1993, eight consecutive pediatric patients with MDS received a marrow transplant from an HLA-identical, mixed lymphocyte culture (MLC) non-reactive sibling. Diagnosis at time of presentation was refractory anemia with excess of blasts (RAEB) in two patients, RAEB in transformation (RAEB-t) in three, and juvenile chronic myelogenous leukemia (JCML, the pediatric counterpart of adult chronic myelomonocytic leukemia) in the remaining three children. Conditioning regimen consisted of busulfan, cyclophosphamide and melphalan, three alkylating agents potentially capable of killing also dormant preleukemic stem cells. The preparative regimen was very well tolerated, and all patients engrafted promptly. Six out of eight patients (75%) are alive and well with a median observation time of 20 months (range 8-34 months). Serial karyotype monitoring and molecular analyses have demonstrated a full chimerism of donor cells and the complete disappearance of trisomy 8 detected before transplant in three cases. All surviving patients have a Karnofsky score of 100%. One boy, affected by RAEB-t with monosomy 7 resistant to treatment with low-dose ara-C, relapsed 11 months after BMT, evolved in AML and died from progressive leukemia. Another patient with RAEB died on day +95 after BMT due to interstitial pneumonia of unclear etiology. This study confirms that allogeneic BMT is the treatment of choice in pediatric patients with MDS, and suggests that the employed conditioning regimen is a safe and effective means for eradicating the preleukemic malignant clone. Particularly noteworthy is that the three children with JCML obtained a complete remission and one of them is now a long-term survivor.
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PMID:Busulfan, cyclophosphamide and melphalan as conditioning regimen for bone marrow transplantation in children with myelodysplastic syndromes. 818 40

A 63-year-old male was diagnosed as chronic myelomonocytic leukemia with normal karyotype in September 1998. He developed acute myelogenous leukemia (AML-M4Eo) in September 2001. The cytogenetic analysis disclosed double t(3;21) at a ratio of 1/20, and the reverse transcriptase-polymerase chain reaction showed AML1/EVI-1 mRNA. He could not achieve complete remission after two courses of induction chemotherapy, and his leukemia cells carrying double t(3;21) were relatively increased. He died of interstitial pneumonia in December 2001. This is the first leukemia case with double t(3;21) and this chromosomal abnormality might play a role in leukemia cell proliferation by generating two AML1/EVI-1 genes.
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PMID:[Double t (3; 21) in acute myelomonocytic leukemia transformed from chronic myelomonocytic leukemia]. 1241 94

Azacitidine is a demethylating and cytotoxic drug for the treatment of adult patients with (1) myelodysplastic syndromes, (2) chronic myelomonocytic leukemia, and (3) acute myeloid leukemia who are not eligible for induction treatment or hematopoietic stem cell transplantation. Widely described in the literature, the main adverse events are hematotoxicity, digestive toxicity, asthenia, cutaneous toxicity, and infections such as neutropenic sepsis and pneumonia. The pivotal phase III comparative and supporting studies did not point out interstitial pneumonitis as a significant adverse event. Rare clinical data from literature report interstitial lung disease secondary to azacitidine administration, which should therefore be considered as a serious potential adverse event. We, herein, report a case of an 86-year-old white woman with acute myeloid leukemia and azacitidine-induced interstitial pneumonitis.
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PMID:Azacitidine-Induced Interstitial Pneumonitis. 2637 47

We describe the case of an 85-year-old man diagnosed with chronic myelomonocytic leukemia whose disease was treated with hydroxyurea for 3 months. He developed respiratory symptoms that were extensively investigated. Despite the intensive treatment, he died of respiratory failure eleven days later. An autopsy revealed diffuse interstitial inflammation of both lungs consistent with drug-induced inflammation. A drug lymphocyte stimulation test was positive for hydroxyurea. Taken together these findings demonstrated that severe interstitial pneumonitis was induced by this drug. Physicians using hydroxyurea must be aware of its potentially life-threatening pulmonary toxicity.
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PMID:Hydroxyurea-induced Pneumonitis in a Patient with Chronic Myelomonocytic Leukemia: An Autopsy Case. 2666 6