Essentials of Diagnosis

Short duration of symptoms, including fatigue, fever, and bleeding. Cytopenias or pancytopenia. More than 20% blasts in the bone marrow. Blasts in peripheral blood in 0% of patients. Classify as acute myeloid leu!emia "#M$% or acute lymphoblastic leu!emia "#$$%.

General Considerations

#cute leu!emia is a hematopoietic progenitor cell malignancy. &ncontrolled proliferation cells and replace normal bone marrow elements. #rise with no clear cause. 'owever, radiation and some to(ins "ben)ene% are leu!emogenic. # number of chemotherapeutic agents "especially cyclophosphamide, melphalan, other al!ylating agents, and etoposide% may cause leu!emia.

General Considerations
Most

of the clinical findings in acute leu!emia are due to replacement of normal bone marrow elements by the malignant cell. $ess common manifestations result from organ infiltration "s!in, gastrointestinal tract, meninges%. #cute leu!emia is potentially curable with combination chemotherapy.

General Considerations

#$$ comprises *0% of the acute leu!emias of childhood. +he pea! incidence is between , and - years of age. .t is also seen in adults, causing appro(imately 20% of adult acute leu!emias. #cute myeloid leu!emia "#M$% is primarily an adult disease with a median age

Symptoms and Signs

Bleeding "usually due to thrombocytopenia% / S!in Mucosal surfaces 0inggival bleeding 1pista(is Menorrhagia $ess commonly, widespread bleeding is seen in patients with disseminated intravascular coagulation "2.C%.

Symptoms and Signs

.nfection is due to neutropenia "falls below% 3004mc$. +he most common pathogens are gram5negative bacteria (Escherichia coli, Klebsiella, Pseudomonas% or fungi "Candida, Aspergillus%. Common presentations include cellulitis, pneumonia, and perirectal infections6 death within a few hours may occur if treatment with appropriate antibiotics is delayed.

Symptoms and Signs

7atients may also see! medical attention / 0um hypertrophy Bone and 8oint pain +he most dramatic presentation is hyperleu!ocytosis, in which a mar!edly elevated circulating blast count "usually 9 200,0004mc$% leads to impaired circulation / 'eadache Confusion 2yspnea

Laboratory Findings

+he hallmar! of acute leu!emia is the combination of pancytopenia with circulating blasts 'owever, blasts may be absent from the peripheral smear in as many as :0% of cases ";aleu!emic leu!emia;%. +he bone marrow is usually hypercellular and dominated by blasts. More than 20% blasts are re<uired to ma!e a diagnosis of acute leu!emia.

Laboratory Findings

'yperuricemia may be seen. .f 2.C is present / +he fibrinogen level will be reduced +he prothrombin time prolonged =ibrin degradation products or fibrin 25dimers present. 7atients with #$$ "especially + cell% may have a mediastinal mass visible on chest radiograph. Meningeal leu!emia will have blasts present in the spinal fluid, seen in appro(imately 3% of cases at diagnosis6 it is more common in monocytic types of #M$. +he #uer rod, an eosinophilic needle5li!e inclusion in the cytoplasm, is pathognomonic of #M$

Laboratory Findings

#M$ classification by ;=#B,; "=rench, #merican, British% was based of morphology and histochemistry / #cute undifferentiated leu!emia "M0% #cute myeloblastic leu!emia "M:% #cute myeloblastic leu!emia with differentiation "M2% #cute promyelocytic leu!emia "#7$% "M,% #cute myelomonocytic leu!emia "M>% #cute monoblastic leu!emia "M3% 1rythroleu!emia "M?% Mega!aryoblastic leu!emia "M-%

Differential Diagnosis

#M$ must be distinguished from other myeloproliferative disorders / Chronic myeloid leu!emia Myelodysplastic syndromes. #$$ must be separated from other lymphoproliferative disease such as / Chronic lymphocytic leu!emia $ymphomas 'airy cell leu!emia. #typical lymphocytosis of mononucleosis and pertussis.

Treatment

+he first step in treatment is to obtain complete remission / normal peripheral blood with resolution of cytopenias normal bone marrow with no e(cess blasts normal clinical status. +he type of initial chemotherapy depends on the subtype of leu!emia.

AML

#M$ are treated with a combination / anthracycline "daunorubicin or idarubicin% plus cytarabine, either alone or in combination with other agents. +his therapy will produce complete remission in *0% of patients under age ?0 years and in 30@?0% of older patients. #7$ is treated differently from other forms of #M$. .nduction therapy should include anthracycline plus all5trans5retinoic acid. Aith this approach 0@ 3% of patients will achieve complete remission.

ALL

#dults with #$$ are treated / 2aunorubicin Bincristine 7rednisone #sparaginase +his treatment produces complete remissions in 0% of patients. Cemission induction therapy for #$$ is less myelosuppressive than treatment for #M$ and does not necessarily produce marrow aplasia

Prognosis

#ppro(imately -0@*0% of adults with #M$ under age ?0 years achieve complete remission. 'igh5dose postremission chemotherapy leads to cure in ,3@>0% of these patients, and high5dose cytarabine has been shown to be superior to therapy with lower doses. #llogeneic bone marrow transplantation is curative in 30@ ?0% of cases. #utologous bone marrow transplantation may be superior to nonablative chemotherapy. Dlder adults with #M$ achieve complete remission in up to 30% of instances. +he cure rates for older patients with #M$ have been very low "appro(imately :0@:3%% even if they achieve remission and are able to receive postremission chemotherapy.