We here report a rare case of allogeneic peripheral blood stem cell transplantation (allo-PBSCT) for an acute myeloid leukemia (AML) patient with the Rh-variant D-- phenotype. People whose red blood cells (RBCs) have a rare deleted Rh phenotype (D--) readily produce anti-Rh17 alloantibodies (an antibody to the RhCc/Ee protein). This causes clinical complications resulting from RBC destruction due to the interaction of alloantibodies with RBCs carrying the corresponding antigen. Consequently, patients with such variants who have formed alloantibodies pose a challenge for transfusions given the scarcity of compatible donors. In fact, in Japan, only 1 in 200,000 individuals are eligible donors. A 26-year-old male AML patient underwent allo-PBSCT from an HLA-matched sibling donor. He had the rare D-- phenotype and developed anti-Rh17 alloantibodies before allo-PBSCT. Given the scarcity of compatible blood donors for transfusions, the patient required autologous blood donations in preparation for allo-PBSCT. RBC engraftment was prompt during allo-PBSCT; thus, no unscheduled RBC blood transfusion was required. Five months later, his blood type changed to that of his sibling donor, and no irregular antibodies to RBCs were detected.
In recent years, fatal cases of primary influenza virus pneumonia have been rare. A 67-year-old woman with secondary myelofibrosis, who had been diagnosed with polycythemia vera 25 years prior, died of primary influenza virus pneumonia. She was immunocompromised due to the underlying disease and ruxolitinib therapy, but she was not vaccinated against influenza. She might have caught the flu from an infected family member. This case reminds us of the importance of infection control measures such as preventing familial infection during ruxolitinib therapy in severely immunocompromised patients.
Chronic myeloid leukemia (CML) is caused by the BCR-ABL oncogene. The Philadelphia chromosome (Ph) from a reciprocal translocation, t(9;22) (q34;q11) causes a fusion gene, BCR-ABL, that encodes a constitutively active tyrosine kinase. Treatment of CML by imatinib is effective to control the tyrosyl phosphorylation of the protein related to the cell signaling. BCR-ABL mRNA is overexpressed in the minimal residual disease (MRD), known as an early sign of relapse. Between December 2005 and June 2008, we measured BCR-ABL mRNA levels in the bone marrow (BM) from patients by quantitative real-time polymerase chain reaction (RQ-PCR) in Aomori Prefectural Central Hospital. Eighty-six samples from 26 patients were collected. Among the 26 CML patients, 11 patients (42%) were in the pretreatment group. Seven (64%) of the 11 patients achieved complete molecular response (CMR). In the post-treatment group consisting of the remaining 15 patients, 9 (60%) patients achieved CMR. The patients receiving imatinib at a dose over 300 mg per day required 13 (6-77) months [median (range)] to achieve CMR. On the other hand, the patients receiving a dose below 300 mg per day required 29.5 (11-84) months [median (range)]. When BCR-ABL mRNA was detected during the treatment course of patients with CMR, careful observation of BCR-ABL mRNA was useful for tracking the clinical course of patients. In conclusion, the BCR-ABL mRNA level was useful for monitoring the clinical course in 26 patients with CML.
Pyomyositis is a purulent infection of skeletal muscle characterized by fever, localized muscle pain and stiffness, swelling and tenderness. Hematological disorder is one of the predisposing conditions for the development of pyomyositis. A 54-year-old man developed methicillin-resistant Staphylococcus aureus pyomyositis during drug-induced pancytopenia. Debridement of the infection foci combined with antimicrobial agents proved effective even in the advanced stage of the disease. In patients with hematological disorders, pyomyositis should be considered when evaluating local myalgia and high fever because this disease can be very difficult to identify and can become rapidly progressive under a myelosuppressive condition.
The Wilms' tumor gene 1 (WT1) encodes a transcription factor that is involved in normal cellular development and cell survival. WT1 mRNA is overexpressed in the minimal residual disease (MRD) of patients with hematopoietic malignancy patients, particularly acute myeloid leukemia (AML). MRD represents the condition with the low levels of leukemia cells in the bone marrow and is known as a sign of recurrence. In hematopoietic malignancies, definition of remission is based on the lack of MRD at submicroscopic level. Between December 2005 and June 2008, we started to measure WT1 mRNA levels in the peripheral blood (PB) from patients by quantitative real-time PCR in Aomori Prefectural Central Hospital. Three hundreds and eight samples from 95 patients were evaluated. The patients included AML (55 patients), acute lymphoblastic leukemia (11), myelodysplastic syndrome (20), malignant lymphoma (5), chronic myeloid leukemia (1), prostatic carcinoma (1), and leukopenia (2). Among the 55 AML patients, 21 patients were pretreated with remission induction therapy. In the clinical course of 21 patients, timely therapeutic approaches could be started for relapse by the early detection of WT1 mRNA overexpression before the morphological findings were apparent. Monitoring WT1 mRNA is helpful to identify patients at high-risk relapse. High overall survival rate (71.2%, 15/21, median: 24.6 months, range 1.1-35.6 months) was achieved in 3 years. The overall survival rate of 34 post-treatment patients was 61.7% (median: 23.5 months, range 0.13-126.5 months after treatment start). In conclusion, the WT1 mRNA level is a sensitive biomarker for monitoring MRD.
