scholarly journals Mixed Phenotype Acute Leukemia Presenting as Leukemia Cutis

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Geetha Narayanan ◽  
M. T. Sugeeth ◽  
Lali V. Soman
Keyword(s):  
Bone Marrow ◽  
Acute Leukemia ◽  
Bone Marrow Aspiration ◽  
Skin Lesions ◽  
The Body ◽  
Leukemic Cells ◽  
Monocytic Differentiation ◽  
Cutaneous Lesions ◽  
Congenital Leukemia ◽  
Leukemia Cutis

Leukemia cutis (LC) is defined as infiltration of the skin by leukemic cells resulting in clinically recognizable cutaneous lesions. It is common in congenital leukemia and acute myeloid leukemia. However, LC has rarely been reported with mixed phenotypic acute leukemia (MPAL). We report the case of a lady who presented with erythematous papular and nodular lesions all over the body. Skin biopsy showed leukemic infiltration and bone marrow aspiration showed MPAL of the T/myeloid with monocytic differentiation lineage. This is the first report of an adult patient with MPAL of the T/myeloid with monocytic differentiation type presenting with leukemia cutis. She was started on chemotherapy with Hyper-CVAD. There is complete resolution of the skin lesions and she has achieved bone marrow remission after the first cycle of chemotherapy.

TREATMENT OF ACUTE LEUKEMIA

PEDIATRICS ◽  
1956 ◽  
Vol 18 (4) ◽  
pp. 643-660
Author(s):  
Joseph H. Burchenal ◽  
M. Lois Murphy ◽  
Charlotte T. C. Tan
Keyword(s):  
Bone Marrow ◽  
Acute Leukemia ◽  
Leukocyte Count ◽  
Bone Marrow Aspiration ◽  
Blood Lead ◽  
Leukemic Cells ◽  
Total Leukocyte Count ◽  
Differential Leukocyte Count ◽  
Complete Replacement ◽  
Susceptibility To Infection

IN TREATMENT of acute leukemia in children, as in treatment of any form of cancer, early diagnosis is of great importance. Although in contrast to many other tumors in children, leukemia is never curable even when detected at the early stages, the sooner treatment can be initiated, the more chance there is of having sufficient time to achieve a beneficial effect. The most important single factor in making the diagnosis of leukemia is a high degree of suspicion. Patients with pains in the bones and joints, with any hemorrhagic tendency, with increased susceptibility to infection or with an unexplained anemia or fever should have an immediate determination of total and differential leukocyte count, hemoglobin, and platelets. If any abnormality is found or if the symptoms persist, a bone marrow aspiration is essential. If these procedures are utilized, it is the rare case in which the diagnosis cannot be made with relative ease. In most cases of acute leukemia, there is almost complete replacement of the normal elements in the marrow by leukemic cells. Depression or absence of erythropoietic activity in the marrow and possibly decreased life span of the circulating erythrocytes leads to severe anemia with the accompanying symptoms of pallor, easy fatigability, and dyspnea on exertion. The decreased megakaryocytic activity in the bone marrow and the decrease of platelets in the circulating blood lead to petechiae, ecchymoses, and other hemorrhagic manifestations. In the peripheral blood the total leukocyte count may be high, normal, or low but, as most of the cells are abnormal in type, the patient is less able to combat infection.

scholarly journals Monocytic leukeima masquerading as a cutaneous lesion

2021 ◽  
Vol 8 (2) ◽  
pp. 293
Author(s):  
Abhishek Singh ◽  
Shifa Farheen ◽  
Preeti Tripathi ◽  
Arijit Sen ◽  
Akriti Kashyap
Keyword(s):  
Bone Marrow ◽  
Skin Biopsy ◽  
Cutaneous Lesion ◽  
Tertiary Care ◽  
The Body ◽  
Leukemic Cells ◽  
Normal Blood ◽  
Induction Phase ◽  
Cutaneous Lesions ◽  
Atypical Cells

Cutaneous lesions preceding a leukaemia are extremely rare and are referred to as ‘aleukaemic leukaemia cutis’. We present a case of acute monocytic leukaemia where skin infiltration of leukemic cells preceded any blood or bone marrow evidence of leukaemia.  A 67-year-old woman presented with multiple cutaneous nodules all over the body of 3 months duration. Cutaneous examination showed multiple erythematous papules and plaques which were present over the face, trunk, extremities and back. Patient was evaluated at a nearby facility and was found to have normal blood parameters (TLC- 7130/cmm, N-57% L-34% M-09%). A skin biopsy done revealed infiltration of the dermis by atypical cells suggestive of a hematolymphoid malignancy. The patient was then shifted to our institution for tertiary care management. The blood counts over a period of one month showed gradually increasing TLC from an initial normal blood count to one showing absolute monocytosis (TLC-25500/cmm, AMC-3825/cmm) and finally abnormally high TLC (TLC-154050/cmm) with 79% monoblasts and promonocytes suggestive of acute leukaemia. A repeat skin biopsy again showed infiltration of atypical cells in the dermis. IHC done showed the atypical cells to be positive for monocytic markers (CD14, CD64). The patient had now started exhibiting systemic findings corroborating with the cutaneous lesions and skin biopsy. Bone marrow aspirate was hypercellular and showed replacement by monoblasts (82%). Cellular morphology was suggestive of AML-M5. Bone marrow biopsy showed a diffuse replacement of marrow by immature cells/blasts. Flow cytometry reports were also positive for monocytic markers. The patient was hence diagnosed as AML M5 with cutaneous metastasis/leukemia cutis and was immediately started on chemotherapy (3+7). Post induction phase, the patient was in remission and her skin lesions subsided. She was subsequently discharged and advised regular OPD follow up for maintenance therapy. This case is reported for its rarity. 

scholarly journals Study on Cutaneous Manifestations of Leukemia and Lymphoma

2015 ◽  
Vol 4 (2) ◽  
pp. 31-34
Author(s):  
Md Abdul Wahab ◽  
AKM Rejaul Haque ◽  
Md Rafiquzzaman Khan ◽  
Biswas Shaheen Hassan ◽  
Md Moksedur Rahman ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hematologic Malignancies ◽  
Skin Lesions ◽  
Cutaneous Lesions ◽  
Cross Sectional ◽  
Cutaneous Manifestations ◽  
Aleukemic Leukemia Cutis ◽  
Leukemia Cutis ◽  
One Year ◽  
Medical University

Leukemia and Lymphoma can present with various cutaneous manifestations. These include specific cutaneous diseases and non-specific cutaneous lesions. Non-specific skin lesions are more common in patients with Hodgkins diseases. Leukemia cutis (specific skin lesions of Leukemia) most commonly occurs concommitent with or following the diagnosis of leukemia. The skin may also be the site of relapse of Leukemia after chemotherapy. Uncommonly leukemia cutis may be identified while the bone marrow and peripheral blood are normal. Those patients are classified as aleukemic leukemia cutis. The objective of this study is to know the prevalence of skin manifestations of hematologic malignancies and to help the diagnosis and management of hematologic malignancies in some extent. It is a cross sectional observational study in which the skin lesions of the patients in hematologic malignancies who were already diagnosed & hospitalized in the hematology department of Bangabandhu Sheikh Mujib Medical University were evaluated in the period of one year. We found malignant infiltrative lesions & hemorrhagic findings both were predominate in leukemia. Infections were predominant in both Leukemia & Lymphoma. CBMJ 2015 July: Vol. 04 No. 02 P: 31-34

Invasive cutaneous cryptococcosis of the nasal planum in a cat – case report

2018 ◽  
Vol XXIII (133) ◽  
pp. 56-62
Author(s):  
Ana Cláudia Balda ◽  
Juliana Cristina Gonçalves ◽  
Renata Cristina Menezes ◽  
Ana Cristina Fascetti de Souza ◽  
Guilherme Durante Cruz
Keyword(s):  
Private Practice ◽  
Infectious Agent ◽  
Skin Lesions ◽  
The Body ◽  
Small Animals ◽  
Cutaneous Lesions ◽  
Mixed Breed ◽  
Deep Mycosis ◽  
The City ◽  
Cutaneous Cryptococcosis

Crytococcosis is the most common deep mycosis in cats, despite its rarity in the medical practice of small animals. The infectious agent of the disease is Cryptococcus sp, an opportunistic fungus that can spread throughout the body. An approximately two-year-old mixed breed cat, weighting 3 kg was presented to a private practice in the city of São Paulo, Brazil. Physical examination relealed pyogranulomatous skin lesions, mainly on the nasal planum and the second digit of the left thoracic lim. The animal presented no neurological or respiratory signs. The response to antifulgal on cutaneous lesions was favorable, but the animal did not resist a second sedation and died within twenty-eight days after starting treatment. This report shows an atypical case of cryptococcosis due to the lack of immunodeficiency.

scholarly journals Expression of the three myeloid cell-associated immunoglobulin G Fc receptors defined by murine monoclonal antibodies on normal bone marrow and acute leukemia cells

Blood ◽  
1989 ◽  
Vol 73 (7) ◽  
pp. 1951-1956
Author(s):  
ED Ball ◽  
J McDermott ◽  
JD Griffin ◽  
FR Davey ◽  
R Davis ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Monoclonal Antibodies ◽  
Acute Leukemia ◽  
Cell Sorting ◽  
Lymphoblastic Leukemia ◽  
Mononuclear Phagocytes ◽  
Leukemic Cells ◽  
Leukemia Cells ◽  
Normal Bone Marrow ◽  
Normal Bone

Monoclonal antibodies (MoAbs) have been prepared recently that recognize the three cell-surface receptors for the Fc portion of immunoglobulin (Ig), termed Fc gamma RI (MoAb 32.2), Fc gamma R II (MoAb IV-3), and Fc gamma R III (MoAb 3G8) that are expressed on selected subsets of non-T lymphocyte peripheral blood leukocytes. In the blood, Fc gamma R I is expressed exclusively on monocytes and macrophages, Fc gamma R II on granulocytes, mononuclear phagocytes, platelets, and B cells, and Fc gamma R III on granulocytes and natural killer (NK) cells. We have examined the expression of these molecules on normal bone marrow (BM) cells and on leukemia cells from the blood and/or BM in order to determine their normal ontogeny as well as their distribution on leukemic cells. BM was obtained from six normal volunteers and from 170 patients with newly diagnosed acute leukemia. Normal BM cells were found to express Fc gamma R I, II, and III with the following percentages: 40%, 58%, and 56%, respectively. Cell sorting revealed that both Fc gamma R I and Fc gamma R II were detectable on all subclasses of myeloid precursors as early as myeloblasts. Cell sorting experiments revealed that 66% of the granulocyte-monocyte colony-forming cells (CFU-GM) and 50% of erythroid burst-forming units (BFU-E) were Fc gamma R II positive with only 20% and 28%, respectively, of CFU-GM and BFU-E were Fc gamma R I positive. Acute myeloid leukemia (AML) cells expressed the three receptors with the following frequency (n = 146): Fc gamma R I, 58%; Fc gamma R II, 67%; and Fc gamma R III, 26% of patients. Despite the fact that Fc gamma R I is only expressed on monocytes among blood cells, AML cells without monocytoid differentiation (French-American-British [FAB]M1, M2, M3, M6) were sometimes positive for this receptor. However, Fc gamma R I was highly correlated with FAB M4 and M5 morphology (P less than .001). Fc gamma R II was also correlated with FAB M4 and M5 morphology (P = .003). Cells from 11 patients with acute lymphoblastic leukemia were negative for Fc gamma R I, but six cases were positive for Fc gamma R II and III (not the same patients). These studies demonstrate that Ig Fc gamma R are acquired during normal differentiation in the BM at or before the level of colony-forming units. In addition, we show that acute leukemia cells commonly express Fc gamma R.

scholarly journals Expression of the three myeloid cell-associated immunoglobulin G Fc receptors defined by murine monoclonal antibodies on normal bone marrow and acute leukemia cells

Blood ◽  
1989 ◽  
Vol 73 (7) ◽  
pp. 1951-1956 ◽  
Author(s):  
ED Ball ◽  
J McDermott ◽  
JD Griffin ◽  
FR Davey ◽  
R Davis ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Monoclonal Antibodies ◽  
Acute Leukemia ◽  
Cell Sorting ◽  
Lymphoblastic Leukemia ◽  
Mononuclear Phagocytes ◽  
Leukemic Cells ◽  
Leukemia Cells ◽  
Normal Bone Marrow ◽  
Normal Bone

Abstract Monoclonal antibodies (MoAbs) have been prepared recently that recognize the three cell-surface receptors for the Fc portion of immunoglobulin (Ig), termed Fc gamma RI (MoAb 32.2), Fc gamma R II (MoAb IV-3), and Fc gamma R III (MoAb 3G8) that are expressed on selected subsets of non-T lymphocyte peripheral blood leukocytes. In the blood, Fc gamma R I is expressed exclusively on monocytes and macrophages, Fc gamma R II on granulocytes, mononuclear phagocytes, platelets, and B cells, and Fc gamma R III on granulocytes and natural killer (NK) cells. We have examined the expression of these molecules on normal bone marrow (BM) cells and on leukemia cells from the blood and/or BM in order to determine their normal ontogeny as well as their distribution on leukemic cells. BM was obtained from six normal volunteers and from 170 patients with newly diagnosed acute leukemia. Normal BM cells were found to express Fc gamma R I, II, and III with the following percentages: 40%, 58%, and 56%, respectively. Cell sorting revealed that both Fc gamma R I and Fc gamma R II were detectable on all subclasses of myeloid precursors as early as myeloblasts. Cell sorting experiments revealed that 66% of the granulocyte-monocyte colony-forming cells (CFU-GM) and 50% of erythroid burst-forming units (BFU-E) were Fc gamma R II positive with only 20% and 28%, respectively, of CFU-GM and BFU-E were Fc gamma R I positive. Acute myeloid leukemia (AML) cells expressed the three receptors with the following frequency (n = 146): Fc gamma R I, 58%; Fc gamma R II, 67%; and Fc gamma R III, 26% of patients. Despite the fact that Fc gamma R I is only expressed on monocytes among blood cells, AML cells without monocytoid differentiation (French-American-British [FAB]M1, M2, M3, M6) were sometimes positive for this receptor. However, Fc gamma R I was highly correlated with FAB M4 and M5 morphology (P less than .001). Fc gamma R II was also correlated with FAB M4 and M5 morphology (P = .003). Cells from 11 patients with acute lymphoblastic leukemia were negative for Fc gamma R I, but six cases were positive for Fc gamma R II and III (not the same patients). These studies demonstrate that Ig Fc gamma R are acquired during normal differentiation in the BM at or before the level of colony-forming units. In addition, we show that acute leukemia cells commonly express Fc gamma R.

scholarly journals CD7+ and CD56+ Myeloid/Natural Killer Cell Precursor Acute Leukemia: A Distinct Hematolymphoid Disease Entity

Blood ◽  
1997 ◽  
Vol 90 (6) ◽  
pp. 2417-2428 ◽  
Author(s):  
Ritsuro Suzuki ◽  
Kazuhito Yamamoto ◽  
Masao Seto ◽  
Yoshitoyo Kagami ◽  
Michinori Ogura ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Acute Leukemia ◽  
Natural Killer ◽  
Nk Cell ◽  
Bone Marrow Involvement ◽  
Leukemic Cells ◽  
Allogeneic Bone ◽  
Disease Entity ◽  
Cell Precursor

Abstract The disease spectrum of natural killer (NK) cell leukemias and lymphomas has recently been expanding with the continuing evolution in diagnostic concepts. We describe here seven cases of acute leukemia of conceivable myeloid and NK cell precursor phenotype in six men and one woman varying from 19 to 59 years of age (median, 46 years). Striking extramedullary involvement was evident at initial presentation, with peripheral lymphadenopathy and/or mediastinal masses. Two lacked any leukemic cells in the bone marrow at diagnosis. Using cytochemical myeloperoxidase staining, less than 3% of the leukemic cells showed positive reactivity. However, expression of CD7, CD33, CD34, CD56, and frequently HLA-DR, but not other NK, T-cell, and B-cell markers was observed. Cytoplasmic CD3 was detected in three of the cases by flow cytometry and in six by Northern blotting, suggesting an origin from common progenitors between the NK cell and myeloid lineages. All but one presented germline configurations of the T-cell receptor β and γ chain genes and Ig heavy chain gene. With regard to morphology, the cells were generally L2-shaped, with variation in cell size, round to moderately irregular nuclei and prominent nucleoli, pale cytoplasm, and a lack of azurophilic granules. Histopathologic examination of biopsied specimens of extramedullary tumors showed a lymphoblast-like morphology, implying the differential diagnostic problem from lymphoblastic lymphomas, especially in cases lacking bone marrow involvement. Three patients were successfully treated with chemotherapy for acute myeloid leukemia (AML), whereas three other patients proved refractory to chemotherapeutic regimens for lymphoid malignancies, although two responded to subsequent AML chemotherapy. However, despite intensive chemotherapy, including allogeneic bone marrow transplantation, most persued fatal courses within 41 months. These data suggested that the CD7+ and CD56+ myeloid/NK cell precursor acute leukemia might constitute a distinct biologic and clinical disease entity. Its recognition appears to be particularly important for the clinicopathologic evaluation of CD56+ hematolymphoid malignancies and the development of therapeutic approaches to such disease.

scholarly journals Extensive Lepromatous Lymphadenitis Preceding Lesions on the Face and Earlobes: An Unusual Presentation of Leprosy in Singapore

2018 ◽  
Vol 10 (1) ◽  
pp. 35-40
Author(s):  
Shi Yao Sam Yang ◽  
Wai Mun Sean Leong ◽  
Cruz Maria Teresa Kasunuran ◽  
Jing Xiang Huang ◽  
Sue-Ann Ju Ee Ho ◽  
...  
Keyword(s):  
Early Stage ◽  
Developed Countries ◽  
Skin Lesions ◽  
The Body ◽  
The Other ◽  
Cutaneous Lesions ◽  
Poor Countries ◽  
Geographical Regions ◽  
Resource Poor ◽  
The Face

Leprosy is also known as Hansen disease, as in some countries the diagnosis of leprosy carries a negative stigma and patients fear being shunned as outcasts. Presently, leprosy is primarily limited to specific geographical regions in resource-poor countries. As a result, there is increased difficulty for the younger generation of physicians today to correctly identify leprosy due to a lack of exposure and a low-index of suspicion, particularly in developed countries. In this case, the indurated lesions over the face demonstrated a preference for the outer lateral aspects over the maxillary areas, the nose bridge, and the pinna of the ears consistent with the organism’s preference for cooler regions of the body. This was also evident in the other skin lesions affecting the more acral regions of the limbs in the early stage of disease progression. There is a need to keep this infective condition as an alternate diagnosis to all unusual cutaneous lesions.

Genomic Analysis of Congenital Myeloid Sarcoma Identifies Significant Bone Marrow Involvement in the Absence of Morphologic Blasts

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 41-41
Author(s):  
Jennifer Kamens ◽  
Jinjun Dang ◽  
Sarah Aldridge ◽  
Jing Ma ◽  
Yuanyuan Wang ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Fusion Gene ◽  
Bone Marrow Involvement ◽  
Pi3k Pathway ◽  
Myeloid Sarcoma ◽  
Whole Genome ◽  
Murine Bone Marrow ◽  
Congenital Leukemia ◽  
Leukemia Cutis ◽  
Targeted Deep Sequencing

Myeloid sarcoma is a tumor mass consisting of blasts at extramedullary sites, most frequently the skin and soft tissues. Skin involvement, also termed leukemia cutis, is a well-described entity in congenital leukemia and is present in approximately 50% of patients diagnosed within the first 4 weeks of life. Most cases of congenital leukemia cutis harbor KMT2A rearrangements, as do up to 80% of cases of infantile leukemia, and are typically accompanied by bone marrow involvement. Here we describe a rare CIC-rearrangement as the driver mutation for a case of congenital cutaneous myeloid sarcoma in a newborn female who was noted at birth to have a violaceous, nodular rash. Skin biopsy of the nodular rash shortly after birth with immunohistochemical staining was consistent with myeloid sarcoma. Bilateral bone marrow aspirates and biopsies were also performed, but failed to reveal an aberrant blast population by morphology and flow cytometry. Cytogenetics of the cutaneous myeloid sarcoma was significant for t(10;19)(q23;q13.2) and fluorescence in situ hybridization performed on the tumor confirmed a CIC-rearrangement. CIC-rearranged sarcomas are a new entity of undifferentiated small round cell sarcoma characterized by fusion events involving the CIC gene but are not a known to be driver mutations in myeloid neoplasms. Despite the absence of a blast population in the bone marrow, 75% of analyzed metaphases carried the t(10;19)(q23;q13.2). To further explore the underlying genomic events, whole genome, exome, and transcriptome sequencing was performed on both sarcoma and bone marrow specimens. Sequencing revealed an in-frame CIC-NUTM2A fusion gene present in both the skin and the bone marrow which has been previously described in a single case of undifferentiated soft tissue sarcoma. There were no large-scale chromosomal losses or gains and no copy neutral loss of heterozygosity events. Two exonic single nucleotide variations (SNVs) were detected, both of which were limited to the skin sarcoma and not predicted to be pathogenic. In vitro and in vivo modeling demonstrated that the CIC-NUTM2A fusion protein was highly leukemogenic. Colony forming assays performed using transduced murine bone marrow revealed that the CIC-NUTM2A fusion conferred self-renewal in contrast to CIC, NUTM2A, and the reciprocal NUTM2A-CIC product which failed to serially replate (p<0.001). Transplantation of CIC-NUTM2A modified murine bone marrow cells resulted in a fully penetrant myeloid leukemia with a median survival of 21 days in primary transplants and 16 days in secondary transplants. Transcriptome analysis of these tumors revealed a distinct gene expression profile when compared to several classic myeloid associated fusion genes including AML1-ETO and MLL-AF6. To further understand the acquisition of a malignant phenotype by morphology between the bone marrow and the sarcoma, targeted deep sequencing was performed for all tier 1, 2 and 3 mutations identified by whole genome and exome sequencing. A shared ancestral clone was identified in both specimens along with three subclones specific to the sarcoma. Four SNVs acquired in the sarcoma were found to be present in regulatory regions of genes that were also differentially expressed between the sarcoma and the bone marrow including C6orf120, SMURF1, TJAP1, and PID1. PID1 was found to be downregulated in the malignant sarcoma specimen and has been previously shown to be a regulator of the AKT/PI3K pathway. Low PID1 expression has been associated with poor outcomes in other malignancies, including pediatric glioblastoma. A genome wide CRISPR screen of our CIC-NUTM2A positive murine leukemia cells revealed an enrichment for PID1 deficient cells as well as PTEN confirming the cooperativity between CIC-NUTM2A and the AKT/PI3K pathway (p=0.009 and p=0.005 respectively). In conclusion, we describe a rare fusion gene, CIC-NUTM2A, which leads to an aggressive myeloid malignancy in both humans and mice. Targeted deep sequencing demonstrated the clonal evolution from the bone marrow and acquisition of a cooperating mutation targeting the AKT/PI3K pathway in a subset of extramedullary cells that led to morphologic transformation. A thorough interrogation of the bone marrow in patients with myeloid sarcoma is warranted even in the absence of morphologic and immunophenotypic blasts by flow cytometry to optimally track residual disease during treatment. Disclosures No relevant conflicts of interest to declare.

Characteristics of De Novo Acute Leukemia Patients with Glycosylphosphatidylinositol (GPI) Protein-Negative Population of Leukemic Cells.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4462-4462
Author(s):  
Hideyoshi Noji ◽  
Tsutomu Shichishima ◽  
Masatoshi Okamoto ◽  
Kazuhiko Ikeda ◽  
Akiko Nakamura ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Leukemia ◽  
De Novo ◽  
Bone Marrow Failure ◽  
Flow Cytometric Analysis ◽  
Leukemic Cells ◽  
Remission Induction ◽  
Color Flow ◽  
Flow Cytometric ◽  
Number Of Patients

Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is considered to be an acquired stem cell disorder affecting all hematopoietic lineages, which lack GPI-anchored membrane proteins, such as CD59, because of abnormalities in the phosphatidylinositol glycan-class A (PIG-A) gene. Also, PNH is one disorder of bone marrow failure syndromes, including aplastic anemia and myelodysplastic syndrome, which are considered as pre-leukemic states. In this study, to know some characteristics of patients with de novo acute leukemia, we investigated expression of CD59 in leukemic cells from 25 patients (female: male=8: 17; mean age ± standard deviation, 57.8 ± 19.5 years) with de novo acute leukemia by single-color flow cytometric analysis. In addition, the PIG-A gene from CD59− leukemic cells sorted by FACS Vantage in 3 patients with acute leukemia was examined by sequence analysis. All the patients had no past history of PNH. Based on the French-American-British criteria, the diagnosis and subtypes of acute leukemia were determined. The number of patients with subtypes M1, M2, M3, M4, M5, and M7 was 1, 14, 2, 4, 2, and 2, respectively. Two of the patients were classified into acute myeloid leukemia with trilineage myelodysplasia from morphological findings in bone marrow. Chromosomal analyses presented abnormal karyotypes in 14 of 25 patients. Flow cytometric analyses showed that leukemic cells from 16 of 25 patients (64%) had negative populations of CD59 expression and the proportion of the populations was 63.3 ± 25.7%, suggesting the possibility that CD59− leukemic cells from patients with de novo acute leukemia might be derived from PNH clones. In fact, the PIG-A gene analyses showed that monoclonal or oligoclonal PIG-A mutations in coding region were found in leukemic cells from 3 patients with CD59− leukemic cells and all of the clones with the PIG-A mutations were minor. Then, various clinical parameters, including rate of complete remission for remission-induction chemotherapy, peripheral blood, bone marrow blood, and laboratory findings, and results of chromosomal analyses were statistically compared between 2 groups of patients with (n=16) and without (n=9) CD59− leukemic cells. The reticulocyte counts (10.5 ± 13.0 x 104/μl) and proportions of bone marrow erythroblasts (17.5 ± 13.9%) in patients with only CD59+ leukemic cells were significantly higher than those (2.5 ± 1.7 x 104/μl, p<0.05; and 5.6 ± 6.2%, p<0.01, respectively) in patients with CD59− leukemic cells. The proportions of bone marrow blasts (69.3 ± 21.1%) in patients with CD59− leukemic cells were significantly higher than those (45.5 ± 19.3%, p<0.02) in patients with only CD59+ leukemic cells. In conclusion, our findings indicate that leukemic cells derived from PNH clones may be common in de novo acute leukemia patients, suggesting that bone marrow failure may have already occurred in localized bone marrow even in de novo acute leukemia.

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