Lymphocytapheresis in a patient with severe aplastic anemia

A 17-year-old Japanese boy with severe aplastic anemia was treated with oxymetholone and prednisolone for 9 weeks without improvement. He then received 2 courses of bolus methylprednisone therapy without improvement and developed a severe liver dysfunction due to this bolus therapy. He had no HLA-compatible sibling for a bone marrow transplantation, and antithymus globulin was not available. A total of 7 lymphocytapheresis treatments were performed, using an IBM 2997 cell separator, over a period of 9 weeks. Hematological and clinical improvement were noted and felt to be related with these lymphocytapheresis treatments, and further therapy was not required. Three years have past since the patient has undergone lymphocytapheresis, and he remains in good health. Thus, lymphocytapheresis may be an alternative therapy for cases of severe aplastic anemia for those patients who have no compatible donor for bone marrow transplantation and when antithymus globulin is not available.     

Severe aplastic anemia associated with seronegative community-acquired hepatitis C virus infection

Summary Aplastic anemia (AA) is a rare complication of viral hepatitis affecting mainly children or young adults. Most reported cases have been associated with community-acquired non-A, non-B hepatitis, but hepatitis A and B have also been implicated in a few patients. We report on a 43-year-old woman with severe AA, in association with acute hepatitis C virus (HCV) infection, diagnosed by detection of HCV RNA by the polymerase chain reaction technique. Her AA was successfully treated with anti-thymocyte globulin and cyclosporin A. The hepatitis C progressed to chronic disease but, despite a follow-up time of 2 years, the patient still has no detectable anti-HCV antibodies, as evaluated with a secondgeneration anti-HCV assay.     

Hypertrophic cranial pachymeningitis in a patient with aplastic anemia

 We report on a 13-year old girl with severe aplastic anemia and hypertrophic cranial pachymeningitis. She was admitted to our hospital with severe headache and vomiting. A computerized tomographic (CT) scan of the brain on the third day of symptoms showed a hyperdense area in the tentorial region. Magnetic resonance imaging (MRI) showed iso-intensity in the same tentorial region in T₁- and T₂-weighted images, and gadolinium enhancement of this region suggested a thickened dura mater. Initially, a diagnosis of subdural or subarachnoid hemorrhage was made. Since her platelet count was low (3000/μl) making the patient a poor-risk candidate for surgery, and the area was limited to the dura mater, conservative therapy, including glycerol administration and platelet transfusion, was carried out. Despite clinical improvement 10 days after admission without specific therapy, the iso-intense region on the left side of the tentorial region remained unchanged on MRI. On the other hand, the iso-intense area on the right side of the tentorial region became hyperdense on T₁-weighted MRI images and was also enhanced by gadolinium. Cerebrospinal fluid findings were normal except for slightly elevated protein at 62 mg/dl. A diagnosis of hypertrophic cranial pachymeningitis of the tentorial dura mater with hemorrhage on the right side was made. Although hypertrophic cranial pachymeningitis is a rare disease, it must be considered in the differential diagnosis of severe headache in a case of aplastic anemia. Received: April 21, 1998 / Accepted: August 28, 1998     

Spontaneous resolution of severe aplastic anemia associated with viral hepatitis a in a 6-year-old child

Recovery from acquired aplastic anemia associated with hepatitis is rare. This case of a 6-year-old boy with severe aplastic anemia is the first reported association of this disease with a hepatitis A infection. Antibody to hepatitis A (anti-HA) was not detected on admission, but was detected three weeks later. Infection with hepatitis B virus, cytomegalovirus (CMV), and Epstein-Barr virus (EBV) were excluded. The peak titer of anti-HA was higher than would be expected for passive transfer of antibody resulting from transfusions. The persistence of antibody for more than 20 months after the last transfusion was not consistent with passive antibody, which would be expected to disappear over that time. This child had indications for allogeneic marrow transplant and a sibling donor who was histocompatible. However, the transplant was postponed because the prognosis was considered to be poor in the presence of active hepatitis. There was a spontaneous remission without the necessity of the transplantation procedure.     

Myocardial infarction caused by Aspergillus embolization in a patient with aplastic anemia

A 38-year-old Japanese man with severe aplastic anemia had invasive pulmonary aspergillosis as a complication. He was treated with amphotericin B for six weeks, but the aspergillosis did not improve. Then he experienced a fatal myocardial infarction. An autopsy revealed disseminated aspergillosis involving pericarditis and Aspergillus embolization to the coronary arteries. This led to the acute myocardial infarction. Cardiac aspergillosis is rare, but should be included within the differential diagnosis when chest pain of unknown origin occurs in an immunosuppressed patient.     

Remarkable improvement of anemia by administration of recombinant human erythropoietin in a patient with aplastic anemia

A 69-year-old female was admitted for pancytopenia. The hematological examination showed leukocytes 1,800/microliters, hemoglobin 5.3 g/dl and platelets 9.6 x 10(4)/microliters. A bone marrow aspiration revealed hypoplasia, but no abnormal cells. Serum erythropoietin titer was 5,100 mU/ml. Diagnosis of aplastic anemia was made. She received 400 ml of blood transfusion twice, and was then treated with recombinant human erythropoietin (rHuEPO) (12,000 U/day) three times a week for eight weeks. Hemoglobin level gradually increased to the level of 12.0 g/dl. This case suggests that there are some cases of aplastic anemia which can respond to treatment with rHuEPO.     

Congenital stomatocytosis associated with aplastic anemia

A seven year-old boy with hereditary stomatocytosis complicated with aplastic anemia was reported. He was admitted to our hospital because of pale and general fatigue. On physical examination, he had severe anemia, petechiae, but no hepatosplenomegaly. Peripheral blood cell count revealed pancytopenia; RBC 103 X 10(4)/microliters, Hb 3.5 g/dl, Ret 21%, WBC 1,200/microliters, Pl 1.3 X 10(4)/microliters, and bone marrow revealed markedly hypocellular marrow. Red cell morphology demonstrated stomatocytosis. Red cell life span (51Cr T1/2) was 12 days, Coombs' test and Ham's test were negative. Indirect bilirubin was 1.1 mg/dl and marked decrease of haptoglobin was found. Family studies showed that his father and sister had stomatocytosis on peripheral blood examination, but no anemia. The patient had severe anemia because of complicated aplastic anemia. Congenital stomatocytosis with aplastic anemia is extremely rare. The authors are interested in a possible relationship between hereditary stomatocytosis and aplastic anemia although the precise mechanism remains to be elucidated.     

Marked reactive plasmacytosis accompanied by drug eruption in a patient with aplastic anemia

A 61-year-old woman with aplastic anemia was admitted to our hospital in October 2009 because of fever and abdominal pain. She had been treated with cyclosporin A without showing any effect. On admission, uterine cancer was diagnosed and the left uterine appendages were swollen. She was treated with cefepime for febrile neutropenia without effect, and left-sided adnexitis was diagnosed. After cefepime was changed to meropenem, marked plasmacytosis was observed in the peripheral blood (23%) and bone marrow (79%) with the appearance of skin eruption. Although the plasma cells were morphologically abnormal, the cytoplasmic immunoglobulin light chain deviation was not detected by flow cytometric analysis, and M protein was not found by serum immunoelectrophoresis. She was diagnosed with reactive plasmacytosis and treated with dexamethasone. The drug eruption and plasmacytosis improved soon after starting the treatment. Although reactive plasmacytosis is observed with a variety of conditions, including infection, neoplasms, autoimmune disorders, and hemolytic anemia, it has not been reported to accompany drug eruption. Reactive plasmacytosis is sometimes not possible to distinguish from plasma cell neoplasms on morphology alone and needs to be diagnosed comprehensively by using flow cytometric analysis and immunohistochemical evaluation.     

Severe aplastic anemia associated with human parvovirus B19 infection in a patient without underlying disease

 Human parvovirus B19 (B19 virus) infection is known to induce aplastic crisis in patients with hemolytic anemia. In healthy subjects, B19 infection may sometimes cause mild pancytopenia, but these changes are transient and recovery is spontaneous. We report the first case of aplastic anemia in a previously healthy boy without any underlying diseases, following asymptomatic infection with the B19 virus. Laboratory examination initially showed thrombocytopenia, mild leukopenia in the peripheral blood, and severe hypoplastic bone marrow. Since pancytopenia developed and worsened progressively, immunosuppressive therapy was given, resulting in a complete remission. Despite the lack of an infectious prodrome, serological and histological analysis revealed an underlying infection with the B19 virus. Thus, B19 virus infection must be considered one of the causes of aplastic anemia in patients without underlying hemolytic anemia and an apparent episode of the viral infection. Received: August 28, 1998 / Accepted: November 18, 1998     

Extramedullary hemopoiesis presenting as a tumor of the posterior mediastinum in a patient with hemolytic anemia

Extramedullary hemopoietic focus is a rare cause of tuberous enlargement of the posterior mediastinum. A 62-year-old man who had been diagnosed as having congenital spherocytic anemia 20 years ago, was admitted to hospital with initial diagnosis of pneumonia. Chest X-ray disclosed inflammatory infiltration in the lower lobe of the right lung and a tumor in the posterior mediastinum. Pneumonia subsided following treatment with antibiotics. Spiral computer tomography with concomitant transthoracic biopsy of the lesion, confirmed the presence of tumor located on the right side of the spinal column, as well as the presence of two minor focci on its opposite side. Cytological examination revealed extramedullary hemopoiesis. Differential diagnosis of tumor in posterior mediastinum, should take into consideration the possibility of extramedullary hemopoietic focci.     

Treatment of platelet-alloimmunization with cyclosporin A in a patient with aplastic anemia

The development of alloantibodies to platelets is a major problem in the supportive management of thrombocytopenia in patients with severe aplastic anemia. We report here a case of aplastic anemia refractory to platelet transfusion. An immunosuppressant, cyclosporin A, which was used for the therapy of aplastic anemia, modulated alloimmunization to platelets in this patient, followed by repeated platelet transfusion. The treatment reduced platelet alloantibodies detected by anti-human immunoglobulin lymphocytotoxicity test, with change of the CD4/CD8 ratio in T lymphocytes in peripheral blood. These results suggest the usefulness of cyclosporin A for the prevention of platelet alloimmunization.     

Allopurinol induced aplastic anemia in a patient with chronic renal failure

A 43 year old female patient with chronic renal failure originated from polycystic kidney disease was admitted with pancytopenia. Prior to the admission, she had a history of taking allopurinol for 3 months. Allopurinol was discontinued immediately and she was treated with blood transfusion (platelet and RBC) and fluoxymesterone. The lymphocyte stimulation tests were negative for allopurinol and oxipurinol. The determination of serum levels of allopurinol and oxipurinol was disclosed to be not so high compared with other patients treated with allopurinol. On 45th day after admission, she was transfused with bone marrow from her elder brother. Thereafter bone marrow finding of the patient began to improve despite the lack of bone marrow engraftment. For further improvement, pulse treatment with corticosteroid was carried out. Although the pathophysiology of allopurinol-induced aplastic anemia remains unknown, it is interesting to note that bone marrow transfusion and pulse treatment with corticosteroid seemed effective in this case.