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Background

Leukemias are neoplastic proliferations of the hematopoietic cells arising within the bone marrow.  As a group, they are classified as acute and chronic.  Acute leukemias have primitive cells called blasts and usually have a rapid and fatal course if untreated.  They are further subdivided into acute lymphoblastic leukemias (ALL) and acute myeloblastic or acute non-lymphoblastic leukemias (AML or ANLL).  Chronic leukemias are proliferations of relatively mature cells and have an indolent course.  Chronic leukemias may transform and convert to acute leukemias with a clone of blast cells.  They, too, are subdivided into chronic lymphocytic leukemias (CLL) and chronic myeloid or myelogenous leukemias (CML).  Pathologists often perform sophisticated analysis of leukemia cells through a combination of flow cytometry and immunohistochemistry.  The newer classification systems are largely the result of discoveries made by pathologists.

Leukemia-Acute Lymphoblastic (ALL)
Leukemia-Acute Myelogenous (AML)
Leukemia-Chronic Lymphocytic (CLL)
Leukemia-Chronic Myelogenous (CML)
Leukemia-Hairy Cell
Leukemia-Prolymphocytic
Myelodysplasia
Myeloproliferative Disorders (Essential Thrombocytosis, Polcythemia Vera, CML)

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/Other Diagnostic Testing  
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

DISEASE ASSOCIATIONS CHARACTERIZATION
RADIATION  


Radiation-induced leukemia risk among those aged 0-20 at the time of the Chernobyl accident: a case-control study in the Ukraine.

Noshchenko AG, Zamostyan PV, Bondar OY, Drozdova VD.

Department of Environmental Sciences, National University Kiev-Mohyla Academy, Kiev, Ukraine

Int J Cancer 2002 Jun 1;99(4):609-18 Abstract quote

A case-control study was conducted to estimate the radiation-induced acute leukemia risk for the period 1987-1997 among residents aged 0-20 at the time of the Chernobyl accident in the most radioactively contaminated territories of the Ukraine (Rivno and Zhytomir regions).

Data were collected on 272 leukemia cases diagnosed between 1 January 1987 and 31 December 1997. Of these, 98 cases were verified and interviewed. Verified cases were compared to 151 randomly selected controls matched by age, gender and type of settlement. The mean value of the estimated accumulated equivalent dose to the bone marrow was 4.5 mSv, and the maximum value was 101 mSv.

A statistically significant increased risk of leukemia was found among males whose estimated radiation exposure was higher than 10 mSv. This association was statistically significant for acute leukemia cases that occurred in the period 1993-1997, particularly for acute lymphoblastic leukemia. A similar association was found for acute myeloid leukemia, diagnosed in the period 1987-1992.

 

LABORATORY AND RADIOLOGY CHARACTERIZATION
FLOW CYTOMETRY  


Flow Cytometric Analysis of Acute Leukemias.

Kaleem Z, Crawford E, Pathan MH, Jasper L, Covinsky MA, Johnson LR, White G.

Departments of Pathology and Immunology (Drs Kaleem, Covinsky, and Johnson and Ms White) and Pediatrics (Dr Crawford and Ms Jasper), Division of Medical Genetics, Washington University School of Medicine, St Louis, Mo; and Department of Pathology, Creighton University School of Medicine, Omaha, Neb (Dr Pathan). Dr Kaleem is currently with the Department of Pathology, Creighton University School of Medicine, Omaha, Neb. Dr Johnson is currently with the Department of Pathology, University of Pittsburgh Medical Center, Presbyterian University Hospital, Pittsburgh, Pa.

 

Arch Pathol Lab Med 2003 Jan;127(1):42-48 Abstract quote

Context.-Acute leukemia displays characteristic patterns of surface antigen expression (CD antigens), which facilitate their identification and proper classification and hence play an important role in instituting proper treatment plans. In addition to enzyme cytochemical analysis, multiparameter flow cytometric analysis has become commonplace in most laboratories for that purpose. The essential role and caveats of flow cytometry in that regard, however, have received little scrutiny.

Objective.-To evaluate the expression of commonly used immunomarkers and patterns in various acute leukemias to help define the best use and role of multiparameter flow cytometry in the diagnosis and proper classification of acute leukemias.

Design.-We have retrospectively analyzed the immunophenotypic data from 508 de novo adult and pediatric acute leukemia patients, as studied using multiparameter flow cytometry in addition to routine morphologic and enzyme cytochemical analysis. Cytogenetic and/or molecular data were correlated in all 41 cases of acute promyelocytic leukemia (APL) and in 203 other cases of acute leukemia where those data were available. We have also determined the positive and negative predictive values of a combined CD34 and HLA-DR expression pattern for the differentiation of APL from other myeloid leukemias.

Results.-In acute myeloid leukemia (AML) other than APL, expression of CD34 was seen in 62% and expression of HLA-DR in 86% of all cases. Twenty-six (10%) of 259 cases of non-APL AML were negative for both CD34 and HLA-DR as opposed to 33 (80%) of 41 cases of APL. None of the cases of APL were positive for both CD34 and HLA-DR in contrast to 149 (58%) of 259 cases of non-APL AML. Fifty-three cases were found to be examples of minimally differentiated AML (AML M0) based on the lack of expression of cytoplasmic CD3 and cytoplasmic CD79a and expression of one or more myelomonocytic-associated antigens and/or myeloperoxidase. Expression of CD20 was seen in 40 (24%) of 168 cases of precursor B-cell acute lymphoblastic leukemia (pB-ALL) and 52 (29%) lacked CD34 expression. Five of 180 cases of pB-ALL and 2 cases of precursor T-cell ALL (pT-ALL) were negative for terminal deoxynucleotidyl transferase (TdT). Aside from cytoplasmic CD3, CD5 and CD7 were the most sensitive antigens present in all 21 cases of pT-ALL. CD33 was more sensitive but less specific than CD13 for myeloid lineage.

Conclusion.-Aside from identification of blasts, flow cytometry was found to be especially useful in the correct identification of AML M0, differentiation of APL from AML M1/M2, and correct identification of TdT-negative ALL and unusual variants, such as transitional B-cell ALL and undifferentiated and biphenotypic acute leukemias.

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
VARIANTS  
LEUKEMIA CUTIS

1-50% of leukemic patients depending upon the type of leukemia

Leukemic infiltration of the skin

May present as macules, papules, palpable purpura, ulcers, erythroderma, and bullous lesions

Leukemia cutis in a patient with chronic neutrophilic leukemia

J Am Acad Dermatol 2001;44:365-9

Case report of a patient with a 2-week history of a pruritic eruption on the arms, hands, and legs

Physical examination revealed red to violaceous plaques on both thighs and knees, in addition to purpuric patches and plaques on the dorsal hands, arms, and legs

Leukemia cutis was demonstrated on biopsy specimens of several lesional sites

The dermal infiltrate consisted of a mixture of immature and mature neutrophilic elements, rather than a predominantly mature neutrophilic dermatosis-the differential diagnosis included Sweet's syndrome

The eruption progressed, despite treatment with topical and systemic corticosteroids

Treatment with systemic chemotherapy did affect partial resolution of the eruption, with parallel decreases in bone pain and white blood cell count, but the disease progressed and the patient ultimately died 5 months after her initial skin findings

Cutaneous promyelocytic sarcoma at sites of vascular access and marrow aspiration. A characteristic localization of chloromas in acute promyelocytic leukemia?

Sanz MA, Larrea L, Sanz G, Martin G, Sempere A, Gomis F, Martinez J, Regadera A, Saavedra S, Jarque I, Jimenez C, Cervera J, de La Rubia J.

Servicio de Hematologia, Hospital Universitario La Fe, Av. Campanar 21, 46009 Valencia, Spain.

Haematologica 2000 Jul;85(7):758-62 Abstract quote

Extramedullary disease (EMD) is a rare clinical event in acute promyelocytic leukemia (APL). Although the skin is involved in half of the reported EMD cases, the occurrence of cutaneous promyelocytic sarcoma (PS) has been described very rarely.

We report here three cases of PS which have the peculiarity of appearing at sites of punctures for arterial and venous blood and marrow samples (sternal manubrium, antecubital fossa, wrist over the radial artery pulse, catheter insertion scar).

At presentation, all patients had hyperleukocytosis and a morphologic diagnosis of microgranular acute promyelocytic leukemia variant confirmed at the genetic level by demonstration of the specific chromosomal translocation t(15;17). A BCR3 type PML/RARa transcript was documented in the two patients for whom diagnostic RT-PCR was available. Patients had morphologic bone marrow remission at the time the PS appeared. A predilection for the development of cutaneous PS at sites of previous vascular damage has been noted, but the pathogenesis remains largely unknown. A potential role for all-trans retinoic acid has been advocated, although one of the three patients in our series had received no ATRA.

A review of the literature revealed six similar cases and hyperleukocytosis at diagnosis was a consistent finding in all of them. A careful physical examination of these particular sites in the follow-up of patients at risk, as well as cutaneous biopsy and laboratory examination of suspected lesions are strongly recommended.

 

HISTOLOGICAL TYPES CHARACTERIZATION
VARIANTS  
GRANULOCYTIC SARCOMA (LEUKEMIA CUTIS) Consider this diagnosis if the cells have a CD43+ only phenotype with CD3- and CD20-
Most sensitive marker is myeloperoxidase
Neutrophil elastase is less sensitive
Chloracetate esterase (Leder stain) is specific but not sensitive-also stains mast cells

Myeloid leukemia cutis: a histologic and immunohistochemical review

Cibull TL, Thomas AB, O’Malley DP, Billings SD.

 


J Cutan Pathol 2008; 35: 180–185. Abstract quote

Background: The histologic diagnosis of myeloid leukemia cutis (LC) can be difficult, requiring confirmatory immunohistochemical stains.

Objective: We reviewed 21 biopsy-proven cases of LC with emphasis on the use of immunohistochemistry in the diagnosis.

Materials and Methods: Clinical and histologic features were reviewed on 21 cases of biopsy proven LC. Immunohistochemical stains for CD4, CD34, CD56, CD68, CD117, CD123, TdT, lysozyme and myeloperoxidase were performed on 12 with available tissue blocks.

Results: Ages ranged from 24 to 88 years (mean = 57), with 12 men: 9 women. Primary hematologic diagnoses included acute myeloid leukemia (n = 14), myelodysplastic syndrome (n = 3), essential thrombocythemia (n = 1) and myeloid leukemia, NOS (n = 3). Monocytic myeloid LC was most common (35%). There was 100% positivity with CD68 and lysozyme. Myeloperoxidase, CD117 and CD34 immunostains were less sensitive in myeloid LC (58%, 33% and 17%, respectively). CD4 was positive in 67%. CD56 was positive in 33%.

Conclusion: Myeloid leukemia with monocytic differentiation more commonly involves the skin than other types of myeloid leukemia. CD68 and lysozyme immunostains, although not lineage specific for monocytes/macrophages, are the most sensitive immunostains in the detection of myeloid LC. Myeloperoxidase immunostains are useful, but immunostains for CD117 and CD34 are insufficiently sensitive. CD4 expression is common, but CD56 expression is not.

Leukemia cutis.

Department of Pathology, The Methodist Hospital, The University of Texas M.D. Anderson Cancer Center, Houston, TX 77030, USA.

 

Am J Clin Pathol. 2008 Jan;129(1):130-42. Abstract quote

Leukemia cutis (LC) is a nonspecific term used for cutaneous manifestations of any type of leukemia. LC has a wide range of cutaneous manifestations, which can make it difficult to clinically distinguish LC from other skin lesions. Patients with LC usually have concomitant systemic leukemia, but occasionally skin involvement precedes the involvement of the bone marrow or peripheral blood. Thus, a skin biopsy can be the first indication of the presence of leukemia in a subset of patients.

The immunophenotyping of routinely processed skin biopsy specimens is very useful in establishing the diagnosis of LC. Although the molecular mechanisms explaining the pathogenesis of LC are not well defined, chemokine receptors and adhesion molecules may have an important role in skin tropism.

We review the literature and recent advances pertaining to LC, with special emphasis on the immunohistochemical assessment and possible mechanisms involved in skin tropism by leukemic cells.
Myeloid Sarcoma Involving the Gynecologic Tract
A Report of 11 Cases and Review of the Literature

Mar Garcia Garcia, MD, etal.
Am J Clin Pathol 2006;125:783-790
Abstract quote
|
Myeloid sarcoma can involve any anatomic site, but involvement of the gynecologic tract is uncommon.

We describe 11 women, 17 to 60 years old, with myeloid sarcoma involving the gynecologic tract, including 5 patients in whom myeloid sarcoma presented as an isolated mass. The uterus was the most frequently involved anatomic site, in 8 patients (5 corpus, 3 cervix). Each neoplasm diffusely infiltrated normal structures, and, cytologically 7 tumors were immature, 3 were differentiated, and 1 was blastic. In 9 cases assessed, immunohistochemical stains showed that all neoplasms were positive for myeloperoxidase and lysozyme; CD117 was positive in 7 of 8 cases, and cytochemical staining for naphthol AS-D chloroacetate was positive in all 6 neoplasms analyzed.

Following chemotherapy, complete remission and long-term survival were achieved in a subset of patients, as was particularly true for 2 patients (cases 8 and 10), with complete remission 12.5 and 31 years after diagnosis, respectively.
Myeloid Sarcoma Involving the Testis

Jose R. Valbuena, MD, Joan H. Admirand, MD, Pei Lin, MD, and L. Jeffrey Medeiros, MD
Am J Clin Pathol 2005;124:445-452 Abstract quote

Myeloid sarcoma is a neoplasm of immature granulocytes, monocytes, or both involving any extramedullary site. Myeloid sarcoma involving the testis, however, is uncommon and very rarely occurs as an isolated mass.

We describe 4 patients with myeloid sarcoma involving the testis, including 2 patients in whom the neoplasm was isolated to the testis (1 unilateral and 1 bilateral). Histologically, 4 neoplasms were poorly differentiated and 1 was blastic. Each neoplasm was shown to be of myeloid lineage, and negative for T- and B-cell specific antigens using immunohistochemical methods. One case was also positive for chloroacetate esterase.

In the literature, most cases of myeloid sarcoma involving the testis represent relapse or the initial presentation of acute myeloid leukemia. Including the 2 cases we report here, only 7 cases of myeloid sarcoma isolated to the testis have been reported.
Myeloid sarcoma of appendix mimicking acute appendicitis.

Palomino-Portilla EA, Valbuena JR, Quinones-Avila Mdel P, Medeiros LJ.

Department of Pathology, Edgardo Rebagliati Hospital, Lima, Peru.
Arch Pathol Lab Med. 2005 Aug;129(8):1027-31. Abstract quote  

CONTEXT: Myeloid sarcoma is a neoplasm of immature myeloid cells involving an extramedullary anatomic site that is usually, although not always, associated with acute myeloid leukemia. Any extramedullary site can be involved by myeloid sarcoma, but involvement of the cecal appendix is uncommon, and symptoms mimicking acute appendicitis as a result of appendiceal involvement are rare.

OBJECTIVE: To describe the clinicopathologic features of 2 patients with myeloid sarcoma involving the appendix who presented with right lower quadrant pain suggestive of acute appendicitis and prompting appendectomy.

DESIGN: Clinical information for both patients was obtained from the medical record. Routine hematoxylin-eosin-stained slides, naphthol-ASD-chloroacetate stain, and immunohistochemical stains for myeloid, B-cell, and T-cell antigens were prepared.

RESULTS: Peripheral blood and bone marrow were infiltrated by coexistent acute myeloid leukemia in case 1 but were negative for leukemia in case 2. In case 2, the patient had a history of acute myeloid leukemia that had been treated by an allogenic bone marrow transplant 7 months earlier. Histologic examination of the appendix revealed poorly differentiated myeloid sarcoma in both cases. Each neoplasm was positive for chloroacetate esterase, myeloperoxidase, lysozyme, and CD43 and was negative for CD3 and CD20.

CONCLUSIONS: Myeloid sarcoma involving the appendix can rarely cause pain or other symptoms mimicking acute appendicitis. A high index of suspicion combined with the use of cytochemical and immunohistochemical studies are helpful in establishing the diagnosis.

Crystalline Inclusions in Granulocytic Sarcoma Report of 2 Cases and Ultrastructural Studies

James A. Strauchen, MD and Ronald E. Gordon, PhD

From the Department of Pathology, Mount Sinai School of Medicine, New York, NY. \

Arch Pathol Lab Med 2002;Vol. 126, No. 1, pp. 85–86. Abstract quote

Two cases of granulocytic sarcoma were found to contain numerous crystalline inclusions identified on hematoxylin-eosin–stained sections as clusters of pointed needlelike crystals present in foci of necrosis or within macrophages. The crystals were negative for chloroacetate esterase and myeloperoxidase. Electron microscopy demonstrated homogeneously dense, bipyramidal structures, indistinguishable from Charcot-Leyden crystals.

Granulocytic sarcomas may contain crystalline inclusions similar to Charcot-Leyden crystals; these structures should be distinguished from crystalline immunoglobulin inclusions occurring in cases of plasma cell myeloma and lymphoplasmacytic lymphoma, which may have a similar appearance.

LEUKEMIA CUTIS

Br J Dermatol 2000;143:773-779
Am J Clin Pathol 1997;107:627-629

Vasculitis common feature with extensive infiltration of the vessel walls


A Case of Acantholytic Dermatosis and Leukemia Cutis: Cause or Effect?

Pamela E. Sakalosky, M.D.; Neil Fenske, M.D.; Michael B. Morgan, M.D.

Am J Dermatopathol 2002; 24(3):257-259 Abstract quote

Leukemia cutis is capable of presenting in a variety of clinical and histologic guises. We describe a 75-year-old man with a recent diagnosis of M0 acute myelogenous leukemia, who presented with multiple pruritic erythematous papules on his chest and back. Microscopically, the epidermis showed acrosyringeal-based acantholysis consistent with transient acantholytic dermatosis (TAD), associated with exocytosis of atypical hematolymphoid cells. In addition, the dermis showed a contiguous atypical hematolymphoid proliferation consistent with conventional leukemia cutis.

To our knowledge, this is the first such case combining features of TAD with leukemia cutis. It remains to be determined whether the acantholysis occurred secondary to the leukemia cutis, was initiated by the migration of leukemic cells, or if the association is merely serendipitous.

 

SPECIAL STAINS/
IMMUNOPEROXIDASE
CHARACTERIZATION
PAX-5  


The value of anti-pax-5 immunostaining in routinely fixed and paraffin-embedded sections: a novel pan pre-B and B-cell marker.

Torlakovic E, Torlakovic G, Nguyen PL, Brunning RD, Delabie J.

 

Am J Surg Pathol 2002 Oct;26(10):1343-50 Abstract quote

Whereas L26 (anti-CD20) is well established as a B-cell marker of high specificity for use in paraffin-embedded tissues and JCB117 (anti-CD79a) is increasingly used, a comparable additional pan-B-cell antibody has hitherto not yet been identified.

Here we have studied the use of a novel anti-pan-B-cell marker Pax-5 for use in diagnostic pathology. Pax-5 encodes for BSAP (Pax-5), a B-cell-specific transcription factor, the expression of which is detectable as early as the pro-B-cell stage and subsequently in all further stages of B-cell development until the plasma cell stage where it is downregulated. Pax-5 is essential for B-lineage commitment in the fetal liver, whereas in adult bone marrow this transcription factor is required for progression of B-cell development beyond the early pro-B (pre-BI) cell stage. Among the B-cell genes that are present in early B-cell development and are upregulated by Pax-5 are CD19 and Igalpha (CD79a).

We have tested a commercially available anti-Pax-5 antibody (anti-BSAP, clone 24) in a series of 592 routinely fixed and paraffin wax-embedded biopsies, including lymph nodes, bone marrow, and various other organs containing lymphoid tissues. Pax-5 protein (BSAP) was detected in all cases of precursor and mature B-cell non-Hodgkin lymphomas/leukemias. In addition, in 97% of classic Hodgkin lymphomas, Reed-Sternberg cells expressed Pax-5. However, Pax-5 was not detected in any of the multiple myelomas, solitary plasmacytomas, and 4% of diffuse large B-cell lymphomas. Among those diffuse large B-cell lymphomas not expressing Pax-5 were only those with terminal B-cell differentiation. All T-cell non-Hodgkin lymphomas, including ALCL and lymphoblastic lymphomas and leukemias, were negative. There was a strong association between Pax-5 and CD20 expression.

We conclude that anti-Pax-5 is an excellent pan-B and pan-pre-B-cell marker. We have found that anti-Pax-5 is superior to anti-CD20 in the diagnosis of pre-B acute lymphoblastic leukemia and classic Hodgkin lymphoma versus ALCL of T and "null" cell type. It was also useful in differential diagnosis between lymphoplasmacytic lymphoma and plasmacytoma. Even though there is an excellent correlation between CD20 and Pax-5 expression, anti-Pax-5 exceeds the specificity and sensitivity of L26 (anti-CD20) because of its earlier expression in B-cell differentiation and its ability to detect all committed B cells, including classic Hodgkin lymphoma.


DIFFERENTIAL DIAGNOSIS CHARACTERIZATION
PERIPHERAL T-CELL LYMPHOMA  

Granulocytic sarcoma: an immunohistologic comparison with peripheral T-cell lymphoma in paraffin sections.

Ritter JH, Goldstein NS, Argenyi Z, Wick MR.

Division of Surgical Pathology, Barnes Hospital, Washington University Medical Center, St. Louis, Missouri 63110.

J Cutan Pathol 1994 Jun;21(3):207-16 Abstract quote

In evaluating histologically malignant infiltrates in the skin, it is often challenging to distinguish granulocytic sarcoma (GS) from selected cases of peripheral T-cell lymphoma (PTCL). These lesions have clinical features in common, in addition to shared histologic attributes. These include similarity in dermal distribution and growth pattern, nuclear characteristics, propensity to recruit other inflammatory cell types, and production of matrical sclerosis.

In order to determine if immunohistology could contribute to differential diagnosis in this setting, we analyzed 15 cases of mucocutaneous GS, and compared them with 11 cases of well-documented PTCL. Antibodies in the CD15, CD20, CD34, CD43, CD45, CD45RO, and CD68 groups were used, as well as anti-myeloperoxidase (anti-MPX), anti-lysozyme (anti-LYSO), Mac387, and MB2.

Anti-LYSO and anti-MPX were sensitive and specific markers of GS, labeling 93% and 80% of GS cases, respectively, and no cases of PTCL. Anti-CD15 and MB2 were also specific for GS, but each labeled only 60% of GS cases. CD34, CD68, and Mac 387 were specific but insensitive markers of GS. CD43 and CD45 were not particularly useful discriminants, with each being seen in 93% of GS cases, but also 64% and 100% of cases of PTCL, respectively. CD45RO was specific for PTCL; it was present in 82% of PTCL cases and no GS cases.

Thus, conjoint reactivity for CD43, CD45, MPX, and LYSO characterizes GS, and differs from the pattern of PTCL, which is characterized by reactivity for CD45 and CD45RO, occasional reactivity for CD43, and lack of other specified markers.

 

PROGNOSIS AND TREATMENT CHARACTERIZATION
CLASSIFICATION

The immunophenotype of 325 adult acute leukemias: relationship to morphologic and molecular classification and proposal for a minimal screening program highly predictive for lineage discrimination.

Thalhammer-Scherrer R, Mitterbauer G, Simonitsch I, Jaeger U, Lechner K, Schneider B, Fonatsch C, Schwarzinger I.

Department of Laboratory Medicine, University of Vienna, Austria.

Am J Clin Pathol 2002 Mar;117(3):380-9 Abstract quote

Bone marrow cells of 325 adults with acute leukemia were immunophenotyped using a panel of monoclonal antibodies proposed by the European Group for the Immunological Characterization of Leukemias (EGIL). Of these, 97.2% could be assigned clearly to myeloid or lymphoid lineage (254 acute myeloid leukemias [AMLs], 48 B-cell lineage acute lymphoblastic leukemias [ALLs], 14 T-cell lineage ALLs), 1.8% as biphenotypic, and less than 1% as undifferentiated.

Immunologic subtyping of ALLs revealed an association between early precursor phenotypes and coexpression of myeloid antigens, particularly CD15/CD65s coexpression and pre-pre-B cell-specific phenotypes and genotypes. The common ALL phenotype was associated with BCR-ABL translocation. Among AMLs, CD2 coexpression was almost exclusively restricted to French-American-British subtypes M3 variant and M4Eo and related molecular aberrations. The most valuable markers to differentiate between myeloperoxidase-negative AML subtypes M0 and ALLs were CD13, CD33, and CD117, typical of M0, and intracytoplasmic CD79a, intracytoplasmic CD3, CD10, and CD2, typical of B cell- or T cell-lineage ALL.

Our results confirm excellent practicability of the EGIL proposalfor immunologic classification of acute leukemias. For myeloperoxidase-negative AMLs, we suggest a scoring system based on markers most valuable to distinguish between AML-M0 and ALLs.

TREATMENT See individual leukemias

Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fifth Edition. Mosby Elesevier 2008


Commonly Used Terms

Auer Rods-These are distinct red rod-like structures present within the cytoplasm of some immature myeloid cells.  When present in many cells, they may indicate a promyelocytic leukemia, a variant of AML. 

Blast Crisis-A transformation of a chronic leukemia to a clinical scenario of an acute leukemia with a proliferation of blasts.

FAB Classification-French-American-British Classification-The most commonly used classification system for acute leukemias.   The system utilizes a combination blast morphology as well as the presence of other blood elements, such as erythroblasts. 

Relapse-Recurrence of leukemia after a period of remission. 

Remission-Eradication of the leukemia cells by chemotherapy or other treatment.

TdT-Terminal deoxytransferase.  An enzyme which is helpful in differentiating ALL cases from AML.  It is present in 95% of cases of ALL but in less than 5% of cases in AML.

Basic Principles of Disease
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Commonly Used Terms
This is a glossary of terms often found in a pathology report.

Diagnostic Process
Learn how a pathologist makes a diagnosis using a microscope

Surgical Pathology Report
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Special Stains
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Last Updated February 25, 2008

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