Rhizomucor variabilis and Hormographiella aspergillata rarely cause human infections. This report details a fatal case of a 14-year-old female with leukemia posthematopoietic cell transplant and relapse with refractory pancytopenia. The patient first developed an R. variabilis var. regularior palate infection and later developed a cutaneous H. aspergillata infection while on posaconazole and caspofungin therapy.
CASE REPORTThe patient was a 14-year-old female with a history of acute myelogenous leukemia diagnosed in July 2007. The patient underwent an allogeneic HLA-matched bone marrow transplant in December 2007 and experienced relapse in April 2008, with a bone marrow aspirate showing a preponderance of blasts, and later failed reinduction chemotherapy. In September 2008, a computed tomography (CT) scan of the chest 2 weeks prior to admission revealed patchy ground-glass opacities with tiny peripheral nodular densities in both lung fields (sparing the left upper lobe) and a 1.2-cm nodule in the right upper lobe. The patient's condition was deemed too fragile to tolerate a diagnostic lung biopsy. She was empirically treated with broad antibacterial and antifungal coverage that included voriconazole. Of interest, the patient also started drinking an herbal tea remedy of an unknown variety. Shortly afterwards, the patient presented with a 2-week history of odynophagia and persistent febrile neutropenia. An examination showed that the patient had white plaques involving the soft palate and pharynx. A smear stained with calcofluor white from a throat culture showed hyphal elements and conidiophores. A biopsy of the palate lesion showed submucosa and mucosa infiltrated with hyphal forms with sparse septation, rare branching, and chlamydoconidia. The patient was started empirically on 800-mg oral (p.o.) doses of posaconazole twice a day (BID). A CT scan of the head and sinuses was negative, while a CT scan of the lungs confirmed pulmonary nodules that had been previously visualized. Serial galactomannan assay results were negative. The fungal isolate obtained in culture from palate biopsy was tentatively identified as a Rhizomucor sp. by the Mount Sinai clinical microbiology laboratory. The isolate was sent to the Mycology Laboratory of the New York State Department of Health for further characterization. The option of surgical debridement was declined by the family due to potentially severe morbidity. At week 2 of therapy, caspofungin was added as an adjunct therapy. By week 2 of therapy, the lesion had decreased in size, with improved symptoms. At week 3 of therapy, she began receiving a new regimen of chemotherapy, along with granulocyte infusions. By week 4, the patient's symptoms had resolved. By week 5 of therapy, the palate lesion was no longer visualized. Throughout, the patient had persistent severe refractory pancytopenia. One and onehalf months after initiation of therapy, she developed an altered mental status and had a generalized seizure. A CT scan of the brain showed multiple hypodense lesions of the c...
Congenital acute leukemia is a rare form of childhood leukemia, in which lineage conversion at relapse is very rarely reported.Here we describe a case of congenital B-cell acute lymphoblastic leukemia (B-ALL) with t(4;11) and t(1;6) translocations, which at relapse underwent a switch to monocytic lineage with persistence of the original cytogenetic translocations and clonal rearrangement of the JH gene. Similar to the other described cases of congenital acute leukemia with lineage conversion, our case had a MLL gene rearrangement and followed an aggressive clinical course.
These results suggest prophylactic AMB is tolerable and may prevent IMI, especially Aspergillus, during the first 100 days post AlloSCT in pediatric and adolescent patients. A randomized study is needed to determine the efficacy of this approach.
Cytopenias are common among pediatric SOT; however, autoimmune cytopenias are infrequently reported. We report five cases of autoimmune cytopenias in pediatric LT patients: two with isolated IgG-mediated AIHA, two with ITP, and one with Evans syndrome (ITP and AIHA). All patients were maintained on tacrolimus as immunosuppression. Viral illness commonly preceded the autoimmune cytopenias. All patients responded well to medical therapy (steroids, intravenous immunoglobulin, and rituximab) and lowering tacrolimus serum level. Prognosis appears to be worse when more than one cell line (e.g., Evans syndrome) is affected, and/or there is no preceding viral illness. A critical literature review of autoimmune cytopenias in children following SOT is conducted. Autoimmune cytopenias are a rarely reported complication of pediatric SOT, but clinicians taking care of pediatric transplant recipients need to be aware of this complication.
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