Bulky lymphadenopathy in acute myeloid leukemia

Two cases of acute myeloid leukemia (AML) presenting with bulky adenopathy are reported. Both patients were febrile at admission and showed massive and diffuse lymph node involvement, hepatomegaly, and splenomegaly. Erythematopapular leukemic skin lesions were present in one case at the onset and developed in the other at the time of relapse. Anemia, thrombocytopenia, and moderate leukocytosis were present in both. The presence of immature cells in peripheral blood and bone marrow allowed a rapid diagnosis of AML, FAB M1, in one patient. In the other case, owing to the paucity of immature cells in peripheral blood and bone marrow, lymph node biopsy with histology, imprint cytology, and immunocytochemistry were essential for the diagnosis (AML, FAB M2, with trilineage dysplasia and basophilic involvement). Both patients achieved complete remission (CR), followed by an early relapse 3 months later. They underwent allogeneic bone marrow transplantation (BMT) from HLA identical siblings. One patient is actually alive and in CR at 6 months after BMT; the other patient showed a leukemic regrowth after transplantation and died 4 months later.     

Lymphoblastic transformation of myelodysplastic syndrome

Mielodysplastic syndromes (MDS) are clonal disorders of the hemopoietic stem cell. About one third of the cases terminate in an acute leukemia, usually acute myeloblastic leukemia. However, few cases of transformation into acute lymphoblastic leukemia (ALL) have been described. We present a case of refractory anemia that transformed into ALL two months after diagnosis and was successfully treated with conventional chemotherapy. Two years later a hyperfibrotic form of MDS was detected in the patient, that soon after terminated in acute megakaryoblastic leukemia. The course of MDS in the present case provides evidence that MDS can involve a pluripotent stem cell, presenting clonal evolution, documented by successive changes in its clinical and hematological features.     

Plasma cell leukemia presenting as a pancreatic mass

A 62-year-old woman presented with a 3-week history of obstructive jaundice. Computed tomography of the abdomen showed marked enlargement of the head of the pancreas and a prominent pancreatic body, suggestive of a neoplasm with associated pancreatitis. The peripheral blood showed an increased number of plasma cells accounting for 50% of the leukocytes. Biopsy specimens of the pancreas, liver, and a peritoneal lymph node showed a diffuse infiltrate of typical and atypical plasma cells (50% of which had cytoplasmic IgG-lambda). Serum and urine protein electrophoresis revealed a monoclonal IgG-lambda spike and Bence Jones-lambda protein, respectively. The bone marrow was diffusely infiltrated by plasma cells. To our knowledge, this is the first reported case of a plasma cell leukemia presenting as a pancreatic mass producing extrahepatic biliary obstruction.     

Frontonasal flap for reconstruction of complete alar defects

BACKGROUND: It is difficult to reconstruct a satisfactory ala. Axial frontonasal flap has been common in reconstruction of nasal tip. We modified this flap to reconstruct nasal ala. OBJECTIVE: A modified axial frontonasal flap was applied for reconstruction of complete unilateral alar defects in two patients. METHODS: Skin from an intact nasal tip covered the alar defect. The resulting defect in the nasal tip was covered with dorsal skin from the nose. Extended mucosa or a hinged nasolabial flap was used to line the mucosal side of the reconstructed ala. RESULTS: The outcome judged by shape, and texture, was satisfactory. This technique can be employed under field block. CONCLUSION: The modified frontonasal flap is one of the ideal techniques to reconstruct an entire nasal ala.     

Adverse skin reactions to the nicotine transdermal system

OBJECTIVE: To evaluate the risk of leukemia associated with congenital abnormalities, a series of matched case-control studies were carried out by the Children's Cancer Group. STUDY DESIGN: Eligible case patients for this analysis included individuals with a diagnosis of leukemia confirmed at a Children's Cancer Group member institution: 2117 diagnosed with acute lymphoblastic leukemia (ALL) and 605 diagnosed with acute myelogenous leukemia (AML). Case patients were compared with matched regional population control subjects selected by using a modified random digit dialing method. Data regarding congenital abnormalities in index children and their siblings were collected by telephone interview with the biologic mother. Relative risk was estimated by using the odds ratio (OR). RESULTS: More congenital abnormalities were reported in index case patients with ALL than in control subjects, with statistically significant increases in multiple birthmarks (OR = 1.35), Down syndrome (OR = 4.85), congenital heart defects (OR = 1.48), and pancreas-digestive tract abnormalities (OR = 2.52). Similarly, birth defects were reported more often among index case patients with AML than control subjects (OR = 2.90), with significant increases in multiple birthmarks (OR = 1.89), Down syndrome (OR = 76.80), mental retardation (OR = 14.47), and congenital heart defects (OR = 2.07). Exclusion of case patients with Down syndrome from the analysis did not change the statistically significant excess of pancreas-digestive tract abnormalities in case patients with ALL or the excess of multiple birthmarks observed in both case patients with ALL and those with AML. For both the ALL and AML analyses, no significant differences in the number of reported congenital abnormalities were seen between siblings of case patients and siblings of control subjects. CONCLUSION: Many of the observed associations with congenital abnormalities occurred in the children with Down syndrome, who are known to have an increased risk for leukemia. The higher reported frequency of birthmarks among case patients may suggest a genetic component to leukemia risk.     

Acute myelomonocytic leukemia in a XYY man

A case of acute myeloid leukemia (M4) in a 29-year-old male with a 47,XYY karyotype is reported. This aneuploidy was found in both bone marrow cells and mitogen-stimulated lymphocytes. Monosomy 7 correlated with myelodysplastic features. The possible role of XYY in increasing the risk of leukemia is discussed.     

Indications, technique and risks in bone marrow transplantation in adulthood

The option of bone marrow transplantation (BMT) significantly improved prognosis of adult patients with hematologic malignancies aged less than 50 years. Allogeneic BMT using the marrow of an HLA-identical family member still provides the most effective method of BMT. Conventional indications for this form of BMT are chronic myeloid leukemia (CML), acute leukemias presenting with adverse risk factors, myelodysplastic syndromes and severe aplastic anemia. If performed early in the disease course (e.g. during the chronic phase of CML or first remission of acute leukemia and MDS) allogeneic BMT cures 50 to 60% of patients. About 20% die of therapy related complications, e.g. graft versus host disease (GvHD), fatal infections or venoocclusive disease of the liver (VOD) and about 20% of patients succumb to relapse of their hematologic disorder. 80% presenting with severe aplastic anemia can be cured, if allogeneic BMT is performed soon after diagnosis without previous immunosuppressive therapy and blood transfusions. BMT with the marrow of a matched unrelated donor or autologous BMT are increasingly used as alternative procedures. A rate of lethal complications as high as 50% hinders rapid extension of BMT with unrelated donors. Therefore, this form of BMT should be restricted to young patients with leukemias, who cannot achieve long-term remission with conventional chemotherapy (in case of acute leukemias) or alpha-interferon (in case of CML). Reconstitution of hematopoiesis is more rapid after peripheral blood stem cell transplantation (PBSCT) compared with autologous BMT. Therefore, PBSCT will replace autologous BMT in most cases. Most favourable results of PBSCT have been reported in patients with malignant lymphomas after relapse or inferior response to primary induction therapy. Due to the higher relapse rate autologous BMT is inferior to allogeneic BMT in leukemia patients. Trials are required to clarify the potential role of myeloablative therapy with stem cell support in the treatment of patients with solid tumors. Many of the preliminary results already published are unsatisfactory and data of larger trials are still lacking. Therefore, BMT or PBSCT cannot be recommended generally for the therapy of patients with solid tumors.     

Acute leukemia presenting as pericardial effusion--a case report

We report a patient with acute lymphoblastic leukemia, who presented with pericardial effusion. There was no haematologic evidence of leukemia at the time of presentation. The pericardial effusion resolved with chemotherapy. Although a common finding at autopsy, clinically evident pericardial effusion is rare in leukemia. It is also extremely rare for pericardial effusion to be the presenting feature or to antedate haematologic evidence of leukemia. Physician awareness is important to make a correct diagnosis.     

Involvement and identification of a lysine in the PPi-site of pyrophosphate-dependent phosphofructokinase from Giardia lamblia

There exists a great deal of overlap between many myelodysplastic syndromes and myeloproliferative disorders. This is most evident in the spectrum of disorders classified under the term chronic myeloid leukemia. These include chronic granulocytic leukemia, atypical chronic myeloid leukemia and chronic myelomonocytic leukemia. Current classification often does not clearly separate these entities since they share many features, both clinically and hematologically. We report here a case that satisfies criteria for both chronic myelomonocytic leukemia and atypical chronic myeloid leukemia, appearing to fluctuate between the two. This lends further evidence for the heterogeneity of these disorders and the need for better definition. An improved classification scheme would allow for more accurate reporting and research into etiology and treatment. The complex cytogenetic abnormalities of the case are unique and to our knowledge have not been reported previously. Also, this case report underscores the importance of cytochemical stains when such disorders are under consideration.     

Acute myeloid leukemia with concomitant expression of T-/B-lymphoid antigens and immunogenotypic alterations exhibiting t(7;12)(q32;q24)

We report here a patient with acute myeloid leukemia (AML) expressing both T- and B-lymphoid-associated antigens. The leukemia cells in this case had rearrangements of not only immunoglobulin heavy-chain but also T-cell-receptor beta- and delta-chain genes. Although a relatively large number of AML patients with lymphoid markers have been reported, only about a dozen AML patients expressing both T- and B-lymphoid markers have been reported so far.     

Report of the first case of invasive fungal sinusitis caused by Scopulariopsis acremonium: review of scopulariopsis infections

Scopulariopsis acremonium is a species of saprophytic fungus not previously reported to cause invasive disease in humans, although invasive infections from other species of Scopulariopsis have been reported and are reviewed. Deep infection with this fungus is associated with a high mortality rate. Invasive fungal sinusitis, in general, is a potentially fatal disease that typically affects immunocompromised patients, such as those receiving intensive chemotherapy or undergoing bone marrow transplantation. We report a case of invasive fungal sinusitis caused by Scopulariopsis acremonium in a patient with leukemia, who was successfully treated with amphotericin B, itraconazole, endoscopic sinus surgery, and granulocyte colony-stimulating factor.     

慢性粒—单核细胞白血病合并Sweet综合征转化为急性白血病一例并文献复习

目的 提高对慢性粒—单核细胞白血病(CMML)合并Sweet综合征的认识。方法回顾性分析1例CMML合并皮肤疱疹患者资料,取皮肤活检进行病理检测,并给予DA、CAG方案等化疗。结果病理诊断为CMML合并Sweet综合征,单用皮质激素治疗效果不佳,CAG方案使CMML获得完全缓解,皮疹得到有效控制。3个月后Sweet综合征复发,CMML进展为急性单核细胞白血病。结论CMML合并Sweet综合征罕见,高度提示短期内进展为急性白血病。     

Congenital leukemia--personal experience

Congenital leukemia is a very rare form of acute leukemia and up to now more than 100 well documented cases have been published. Symptoms and signs of the disease may be faintly differentiated and be a differential diagnostic problem for a long period of time in comparison to other common diseases of the newborn. This is a case report on two newborns with congenital leukemia. It was an acute non-lymphoblastic leukemia in the first case, and acute lymphoblastic leukemia in the second. In both cases there were nonspecific manifestations of the disease (no weight gaining, hemorrhagic enterocolitis). In both cases polyhemotherapeutic protocols were performed. In the first case only partial remission occurred and death occurred during the hemathologic recidive of the disease. In the second case, 15 months after the diagnose of congenital leukemia has been established, complete remission is in course.     

Hand-mirror cells acute lymphoblastic leukemia (L3)

To our knowledge, this report represents the first confirmed case in Japan of a 15-year-old boy with acute lymphoblastic leukemia (ALL).L3 with hand-mirror cells (HMC) in the bone marrow. HMC lymphoid leukemia is an unusual variant of ALL in which the bone marrow lymphoblasts manifest distinctive hand-mirror morphologic features. HMC lymphoblast is characterized by an asymmetric foot-like cytoplasmic process that extends from the portion of the cell, thus giving it the light-microscopic appearance responsible for its name. Besides ALL, HMC has been reported in acute myeloblastic leukemia (AML), blastic crisis of chronic myelogenous leukemia, non-Hodgkin's lymphoma, and infectious mononucleosis. HMC has been reported to be prevalent in ALL.L1 and L2 as compared with L3.     

Pre-ECT cardiology consultation

A case of multiple primary Carcinomas of the gastrointestinal tract is reported in a man presenting with Carcinomas of the rectum and oesophagus.     

A sole del(15q) anomaly in post-myelodysplasia acute myeloid leukemia

We report the second case of post-myelodysplasia acute myeloid leukemia (post-MDS AML) with a sole chromosome change del(15q). This anomaly is rarely seen. To our knowledge, only seven cases so far have been reported in human neoplasias, including one case each of acute myeloid leukemia (AML), acute lymphoid leukemia, post myelodysplasia AML, myelodysplastic syndrome, myelofibrosis, macroglobulinemia, Hodgkin's lymphoma and uterine leiomyoma. This case suggests that del(15q) is related to lympho-myeloproliferative disorders. Moreover, we speculate that certain oncogene(s) located on 15q might have some role in the progression of the disease, since the del(15q) anomaly appeared only in the AML phase in this case.     

Case report: massive cardiopulmonary cysticercosis in a leukemic patient

A 53-year-old woman who had acute leukemia and massive cardiopulmonary cysticercosis is reported. Pulmonary cysticercosis is rare and in this case may have been promoted by the immunosuppression caused by acute leukemia.     

Cutaneous involvement in chronic myelomonocytic leukemia

BACKGROUND: Chronic myelomonocytic leukemia has been associated with various nonspecific cutaneous manifestations. Rarely has the leukemia been reported to directly affect the skin. METHODS: This case documents the progression of a patient who ultimately developed chronic myelomonocytic leukemia, by clinical examination, hematologic parameters, dermatopathology, and bone marrow pathology. RESULTS: The skin showed nonspecific cutaneous involvement, progressing to specific leukemic lesions parallel with increasing systemic and hematologic involvement. CONCLUSIONS: Chronic myelomonocytic leukemia can manifest with lesions of leukemia cutis. The possibility of nonspecific cutaneous involvement in the preleukemic phase exists.     

Otoneurological manifestations in Chiari-I malformation

The type I Chiari malformation consists of a caudal displacement of the cerebellar tonsils through the foramen magnum into the cervical spinal canal. The most common presenting symptoms, such as pain, weakness and headache, are frequently preceded by otoneurological symptoms. Sensorineural hearing loss, vertigo, nystagmus, dysequilibrium, tinnitus and other cranial nerve involvement have been reported in Chiari-I malformation. A case report is presented and the clinical features of the disease are discussed with emphasis on the otoneurological aspects.     

Comments on chronic environmental cadmium toxicosis in horses and cattle

We report a case of postanginal septicaemia complicated by bronchopneumonia and haemophagocytosis in a 19-year old male, presenting with severe thrombocytopenia. We believe that this is the first reported case of thrombocytopenia due to haemophagocytosis in this unusual condition.