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A 15-years-old boy presented with one day history of bleeding gum, subconjunctival bleed and purpuric rash. Investigations revealed followed results: Hb- 6.4 gm/dl; TLC- 26500/mm3, platelet 35,000 mm3; PT -20 sec with a control of 13 sec; PTT- 50sec; and Fibrinogen 10mg/dL. Peripheral smear was suggestive of AML. Which of the following is the most likely? (AIIMS May 2010)

A Myeloblastic leukemia without maturation

B Myeloblastic leukemia with maturation

C Promyelocytic leukemia

D Myelomonocytic leukemia

Ans. C

Promyelocytic leukemia

AML (FAB Classification) (Ref. Robbins 7th ed pg. 693)

M0 Minimally differentiated AML – Blasts myeloperoxidase negative

M1 AML without maturation – Very immature, only 3% peroxidase positive

M2 AML with maturation – most common subset, associated with t(8;21)

M3 Promyelocytic – High incidence of DIC, many Auer rods per cell patients are younger associated with(t15;17)

M4 Myelomonocytic – monoblasts are positive for nonspecific esterases, associated with the inv(16)

M5 Monocytic – occur in older patients

M6 Erythroleukemia – makes up 1% of de novo AML and 20% of therapy related AML.

M7 Megakaryocytic leukemia – Myelofibrosis seen in most cases.

Pathology of AML

1. AML blasts contain azurophilic granules

2. AML blast do not contain PAS positive material.

3. Terminal deoxy transferase (TdT) is present in 95% cases.

4. Auer rods are presents

AML - Usually occurs >40yrs, it is uncommon below 40 years.

Condition predisposing to AML

1. Polycythemia Vera, Essential Thrombocytosis, CML, Myelofibrosis 2. Myelodysplasia 3. Benzine

Clinical features

1. Patient has fever, anemia, bleeding tendency, infection and sternal tenderness.

2. Rarely patients may present with symptoms from a mass lesion located in the soft tissues, breast, uterus, ovary, cranial or spinal dura, gastrointestinal tract, lung, mediastinum, prostate, bone, or other organs.

3. The mass lesion represents a tumor of leukemic cells and is called a granulocytic sarcoma, or chloroma.

4. Typical AML may occur simultaneously, later, or not at all in these patients.

5. This rare presentation is more common in patients with t(8;21). {Ref. Hari- 17th - Page 679}

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