A 59-year-old man presented with a painless testicular mass and underwent a radical orchiectomy. The resected specimen showed a 5-cm-sized, white-yellow and homogenous solid mass in the testicular parenchyma. Histologically, the central part of the tumor exhibited typical features of seminoma. The peripheral part of the tumor exhibited diffuse infiltration of small, monotonous lymphoid cells involving the tunica albuginea. The monotonous lymphoid cells were immunoreactive for CD20, CD79a, CD5, and CD23, and negative for CD3, CD10, and cyclin D1. Kappa light chain restriction was detected on flow cytometry using the resected specimen. Considering the circulating lymphoid cell count of >5.0×103/µL, we diagnosed the peripheral component of the tumor as an infiltration of chronic lymphocytic leukemia. This extremely rare combination of seminoma and lymphoid neoplasm should be considered in the differential diagnosis of classic seminoma with extensive lymphoid reaction in tumors arising in elderly patients.
Herein we describe a rare case of acute appendicitis associated with localized aspergillosis in an 8-year-old boy with acute lymphoblastic leukemia. During chemotherapy, the patient complained of mild abdominal pain in the peri-umbilical area and displayed an increased C-reactive protein level. Abdominal ultrasonography disclosed appendicitis and consequently an appendectomy was done.
Histologically, acute appendicitis and Aspergillus hyphae were identified in the lumen and necrotic mucosa. However, there was no evidence of systemic aspergillosis. While aspergillosis is a common fungal infection in immunocompromised patients treated with chemotherapy, acute appendicitis associated with localized aspergillosis without systemic infection is a very rare occurrence.
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We report here on a rare case of a patient who presented with an extramedullary B lymphoblastic crisis as an initial manifestation of chronic myelogenous leukemia (CML). A 71-year-old man visited the emergency room due to suddenly developed dysarthria and right side weakness. Emergency craniotomy was done under the presumptive diagnosis of subdural hemorrhage. During the operation, a poorly demarcated firm mass was identified in the leptomeningeal space. Microscopically, the majority of the tumor was composed of monotonous immature cells with blast morphology, and these cells were immunoreactive for TdT, CD34, CD10 and CD20, indicating the precursor B-cell phenotype. The peripheral area of the tumor consisted of myeloid cells in various stages of maturation, and these cells were reactive for myeloperoxidase, chloroacetate esterase, CD43 and CD15.
FISH analysis using the LSI bcr-abl dual color probe showed gene fusion signals in both the B-lymphoblasts and myeloid cells. The peripheral blood and bone marrow findings were consistent with CML with no evidence of a blast crisis.
Cytogenetic study of the bone marrow demonstrated the 46, XY, t(9;22)(q34;q11) chromosome. A diagnosis of extramedullary B lymphoblastic blast crisis in a patient with Philadelphia chromosome-positive CML was made. Despite treatment, the patient died 3 months after he was diagnosed.
Herein, we describe cases of pulmonary acute graft-versus-host disease (aGVHD) in two patients occurring after allogeneic hematopoietic stem cell transplantation (HSCT) due to precursor B-cell acute lymphoblastic leukemia in a 6-year-old patient and in acute myeloid leukemia in a 14-year-old boy. In both cases, chest CT revealed confluent ground-glass attenuation along the bronchovascular bundles, as well as some bronchial dilatation. Microscopically, in both cases we noted a characteristic bronchiolocentric pattern and bronchiolar epithelial changes, which included denudation of the bronchiolar epithelium, regenerating atypical cells, and wall thickening with subepithelial or transmural fibroblast proliferation, along with some lymphocytic infiltration. One patient died on day 86 after allogeneic HSCT due to sudden acute respiratory distress syndrome, and the other patient currently remains alive without active aGVHD. The authors' experiences in these two cases demonstrate that intense awareness of the pathologic findings of GVHD is mandatory after allogeneic HSCT.
Although chronic myelogenous leukemia (CML) may be involved in any part of the body, infiltration of the body fluid has rarely reported in the literature. Here we report on a 35 year-old male patient who was diagnosed chronic myelogenous leukemia ten years previously and he received allogenic hematopoietic stem cell transplantation. He then presented with left knee pain eight years after the initial diagnosis.
MRI revealed a soft tissue mass at the distal femur.
Cytology of the joint fluid revealed myeloblasts, promyelocytes, eosinophilic myelocytes, band neutrophils, megakaryocytes and orthochromatic erythroblasts, which was all consistent with leukemic infiltration of the knee joint fluid. The immunohistochemistry was positive for CD34, CD117 and myeloperoxidase (MPO). Despite that the patient underwent radiation therapy, MRI revealed growth of the mass, and ten months later, the lymphoid blast phase of CML was confirmed after biopsy. The patient received an above knee amputation. Five months later, multiple masses were revealed on PET-CT at the left iliopsoas muscle, abdominal wall and bones. Bilateral pleural effusion occurred shortly after this. Cytologic evaluation of the pleural fluid also revealed blast-like cells, and histologic evaluation of the abdominal mass confirmed the lymphoid blast phase of CML with positivity for CD3, UCHL-1, CD34 and CD117, but negativity for MPO.
Hee Jung Lee, Young Shin Kim, Yong Gu Kim, Kyung Ja Han, Kyo Young Lee, Chang Suk Kang, Sang In Shim, Jong Wook Lee, Woong Shick Ahn, Soo Pyung Aim, Seung Il Kim
Prolymphocytic leukemia is a chronic lymphoproliferative disorder, characterized by prominent splenomegaly, prolymphocytes accounting for more than 55% of circulating lymphocytes, no significant peripheral lymphadenopathy and short term survival with terminal fatal multi-organ failure.
We report a case of B-cell prolymphocytic leukemia in a 57-year-old woman who presented with easy bruising and arthritis for 1 year and low abdominal pain for 2 months.
Physical examination revealed gingival hypertrophy and mild splenomegaly. On peripheral blood smears the leukocytes were markedly increased in number due to leukemic cells that count about 62% of leukocytes. The bone marrow aspiration smear and biopsy revealed diffuse infiltration of medium to large prolymphocytes having moderate amount of basophilic cytoplasm, round to oval nuclei with coarse chromatin, and prominent nucleoli. Abdominal pain aggravated despite chemotherapy, and pelvic computed tomography (CT) revealed a huge lobular pelvic mass which had increased in size on the follow-up CT. Total hysterectomy with bilateral adnexectomy was performed. Microscopic findings included massive infiltration of prolymphocytic cells in the uterus, upper vaginal wall, bilateral ovaries, and bilateral mesosalpinges. On immunohistochemistry, the leukemic cells showed B cell gamma light chain phenotype.
JL1 is a novel human thymocyte differentiation antigen, which is exclusively expressed by double positive (CD4+ CD8+) cortical thymocytes. We previously reported that the JL1 antigen was selectively expressed on the surface of acute lymphoblastic leukemia cells.
T-Lymphoblastic lymphoma/acute lymphoblastic leukemia (T-LBL/ALL), the 6th prevalent lymphoma in Korea, is composed of immature neoplastic lymphoid cells and shows a rapid response to appropriate treatment. Early and precise diagnosis of LBL/ALL is crucial. Light microscopic distinction of LBL/ALL from other non-Hodgkin's lymphomas can sometimes be difficult and is aided by immunophenotypic studies. This study is designed to investigate the diagnostic utility of anti-JL1 monoclonal antibody (YG5) for LBL/ALL in formalin fixed, paraffin embedded tissue. We collected 25 cases of LBL/ALL (18 T-cell, 5 B-cell and 2 undetermined lineage) from 1993 through 1998. We confirmed the diagnosis using morphologic and immunophenotypic data. Strong JL1 expression along cell membrane was observed in 16 out of 18 T-LBL/ALL cases (89%). In 28 cases of other types of lymphomas of including 7 cases of non-T LBL/ALL and 14 cases of small round cell tumors, no JL1 expression was identified. These results show that the immunostaining for JL1 using YG5 on paraffin embedded sections can be useful for the specific diagnosis of T-LBL/ALL in routine diagnostic service.
Hairy cell leukemia is an uncommon lymphoreticular disorder which primarily involves bone marrow, spleen, and peripheral blood. Patients, mostly men, present with splenomegaly and pancytopenia usually. A 62-year-old man was admitted with an abdominnal mass which had grown slowly for 20 years. On physical examination, an enlarged spleen was palpated without tenderness. An abdominal CT scan showed a diffusely enlarged spleen, which measured 20 cm in greatest dimension.
In the peripheral blood, many atypical lymphocytes with abundant, delicate, surface projections were noted. They had tartrate-resistant acid phosphatase(TRAP) activity.
Thrombocytopenia (60,000/mm3) was observed in the complete blood counts, Other laboratory data were within normal limits. He underwent splenectomy. The submitted spleen measured 26x15x5 cm and weighed 2150 gm. It was well encapsulated and the outer surface was smooth. Cut surfaces were diffusely dark-red. White and red pulps were indistinct. There was no mass-like lesion. Microscopically, the spleen consisted of monotonous mononulcear cells which involved red pulp. The white pulp was diminished, and could be barely recognized. Cells had small round nuclei and abundant cytoplasm. Ultrastructurally, cells with numerous slender surface projections were noted. In Korea, hairy cell leukemia is exceedingly rare. We report a case of hairy cell leukemia with characteristic pathologic features of spleen as well as those of peripheral blood.
Acute hybrid leukemia is an uncommon disease that demonstrates malignant transformation expressing lymphoid and myeloid cell lineage. We experienced a case of 25-year-old man with acute leukemia with unusual characteristics: unclassifiable morphology and undifferentiated cytochemistry by French-American-British (FAB) criteria. Microscopically, it disclosed monotonous tumor cell population in lymph node with vascular plugging and perivascular infiltration, and indian file appearance in capsule and surroun ng adipose tissue. Results of flow cytometry and immunohistochemical studies of frozen sections, cytospins, and formalin fixed lymphoid tissues disclosed hybrid form characterized by myeloid and lymphoid cell lineage. The immunophenotype analysis showed both anti-T cell, anti-B cell and anti-myeloid cell monoclonal antibody reactivity; blast cells were consistently CD7+(94.6%), CD13+(97.1%), and CD19+(85.22%). The normal hematopoietic cells were almost replaced by tumor cells in PB and bone marrow. In preparation of cytospin of peripheral blood(PB) cells separated by a Ficoll-hypaque gradients, blast cells were negative for Sudan black B, myeloperoxidase, periodic acid Schiff, and nonspecific esterase.
Granulocytic sarcoma is a rare localized tumor composed of granulocytic precusor cells. Granu-locytic sarcoma occurs in a variety of clinical conditions and it is often misdiagnosed histologically. Differential diagnosis frorh lymphoma or nonhematopoietic malignancies such as undifferentiated carcinoma or sarcoma is difficult in the routing histologic examination. An evaluation of clinical and histopathologic features was done on 4 cases of granulocytic sarcoma which were diagnosed at Pusan Paik Hospital from 1988 to 1992. During the period, 282 cases of myelogenous leukemia were diagnosed. Immunohistochemical reaction for lysozyme, myelopero-xidase, leukocyte common antigen, epthelial membrane antigen and cytokeratin was assessed comparing to lymphoma and undifferentiated carcinoma. The histologic features of the granulocytic sarcoma revealed thin nuclear membrane, fine chromatin pattern and one or two small nucleoli. It also often involved the vascular wall and infiltrated the native structures without destruction. Immunohistochemical stain revealed that all(4 cases) of granulocytic sarcoma showed diffuse and strong positivity for myeloperoxidase, and partial but strong positivity for lysozyme. One case of granulocytic sarcoma was negative and 3 cases revealed focal positive reaction for LCA, and all 4 cases was negative for cytokeratin and EMA. In summary, careful observation under light microscopy with immunohistochemical stain for myeloperoxidase, lysozyme, and LCA is helpful in the differential diagnosis of granulocytic sarcoma from malignant lymphoma and cytokeratin and EMA is useful for differential diagnosis from undifferentiated carcinoma.
In view of the possible role of portal circulation in hematogenous spread of Candida species, a case of hepatic candidiasis occurred in an eight-year-old child with acute lymphoblastic leukemia (ALL) treated by chemotherapy is presented. Symptoms and signs referable to the hepatic disease in this patient included hepatomegaly, icteric sclera and abdominal pain. There were no particular manifestations suggestive of deep mycotic involvement of any sepcific organs or tissues other than the liver. Culture of the blood was negative for one month. On the 24th hospital day the patient died with the presumptive diagnosis of ALL, disseminated intravascular coagulation, acute renal failure, pulmonary edema, cholecystitis and oral thrush. A needle necropsy was performed and revealed fungal aggregates replacing the large foci of hepatic cell loss. It is suggested that, when the gastrointestinal tract serves as the portal of entry, the liver could be the visceral organ involved first in the course of disseminated candidiasis.
Leukemia is a rare disease in the newborn infant. We have presented an autopsy case of congenital acute myelocytic leukemia in a female neonate and discussecd with review of literature. At birth, she was relatively in good health with 4.2 kg in body weight except a large cephalhematoma on left parietal scalp and multiple subcutaneous nodules with ecchymosis on entire body surface. Hemoglobin concentration was 12.0 gm/, Hct 34.6% and erythrocyte count was 2.24 millions. Of 212,400 leukocytes/mm2, 47% were myeloblast.
Biopsy of skin nodules reveal leukemia cutis, which disappear dramatically with anticancer drug. The infant was expired 12 days after admission due to intracerebral hemorrhage and acute renal failure.