leukemia
DESCRIPTION
LEUKEMIA. ↑ leukocytes Acute leukemias 1. Acute Lymphoblastic Leukemia (ALL) 2. Acute Myelogenous Leukemia (AML). Chronic leukemias 1.Chronic L ymphoblastic Leukemia (CLL) 2. Chronic Myelogenous Leukemia (CML). Leukemias. Acute Leukemias. Blast predominate Child or elder - PowerPoint PPT PresentationTRANSCRIPT
LEUKEMIA
Leukemias ↑ leukocytes
Acute leukemias
1. Acute Lymphoblastic Leukemia (ALL)2. Acute Myelogenous Leukemia (AML)
Chronic leukemias
1.Chronic Lymphoblastic Leukemia (CLL)
2. Chronic Myelogenous Leukemia (CML)
Acute Leukemias Blast predominate Child or elder Short & drastic course ALL – Lymphoblasts (pre-B or pre-T) AML – Myeloblasts
Chronic Leukemias More mature cells Middle age Longer & less devastating course CLL – Lymphocytes CML – Myeloid stem cells
Acute Leukemias accumulation of blasts in the marrow
Acute Lymphoblastic Leukemia (ALL)
Children Lymphoblasts (pre-B or pre-T) Neoplastic transformation of the
lymphoid stem cells• Progressive accumulation of
Lymphoblasts in the bone marrow
• Suppression of normal hemopoiesis
ALL
• Primarily a disease of children and young adults
• B-cell subtype (80%)• T-cell subtype (20%)
ALLAbrupt onsetSigns and symptoms related to:
• Suppression of normal hemopoiesis
bone pain and tenderness• Tissue infiltration ( megaly)
(lymph nodes, spleen, liver, testicles, CNS)• Mediastinal mass ( T-ALL ) thymus involvement
Headache, vomiting, nerve palsies from meningeal spread.
Signs &Symptoms Anemia Infection Bleeding Bone pain Arthritis Splenomegaly Lymphadenopathy CNS involvement
ALL - PROGNOSISFavorable: Unfavorable:Pre-B T or mature B Age 3-7 < 1, > 10WBC < 10 000/uL > 50 000/uLFemale MaleMinimal organ Prominent
organinvolvement involvement
ALLPrognosis:
• Age 3-7, pre-B, > 90% - CR ( 2/3 - cure )
• Adults, mature B and T-ALL:Less favorable
Therapy: Chemotherapy supportive care, BMT
ACUTE MYELOGENOUS LEUKEMIA ( AML )
- Adults- Myeloblasts- monoblasts, eosinoblasts,
megakarioblasts, proerythroblasts, basophiloblasts
- Auer rods in the cytoplasm of the cells- Very rapidly progressive malignancy
AML
Auer rods in AML
Differentiation from ALL may be made by microscopy – presence of Auer Rods.
Can also be made based on immunotyping and molecular methods.
Clinical features based on Marrow failure –anemia, bleeding,
DIC, infection… Leukemic infiltration – bone pain,
CNS signs, hepatosplenomegaly, lymphadenopathy…
Constitutional upset -- malaise, fever, weakness, polyarthritis.
Course:• Rapidly fatal if untreated (< 2 mo )• Median survival - 3 years after chem..• Adverse prognostic factors:
Age > 60 Previous chemotherapyLeukocytosis > 100,000 /ul
Therapy: Chemotherapy, supportive, BMT
***Remember this*** For Acute leukemias
acute leukemias = too many blasts in the marrow
2 broad categories: AML vs. ALL a hematologic urgency prognosis is poor in adults; but good
in kids with ALL.
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
A monoclonal lymphoproliferative disorder characterized by lymphocytosis(>4000/cu.mm),lymphadenopathy and splenomegaly
B - CLL > 95%T - CLL
CLL
• Most common adult leukemia in Western society (30% of all leukemias)
• Monoclonal proliferation of the small lymphocytes…
• Age > 40 M:F / 2:1
CLL - PathologyBlood:
• Lymphocytosis( > 10,000 u/L - diagnostic )
• (+) Coombs test (20%)• Hypogammaglobulinemia (50-70%)• Anemia, thrombocytopenia, neutropenia
Bone marrow:• nodular / interstitial infiltrates• diffuse - obliteration of normal hemopoiesis
Lymphadenopathy, Hepatosplenomegaly(50-60%)
CLL - Clinical course
Initially: asymptomaticAdvanced disease:
• bacterial infections, hemorrhage
Prognostic factors:• extent of tumor burden• pattern of marrow infiltration• chromosomal abnormalities
Median survival: ~ 6 years
CLL - Course
• CONVERSION TO PROLYMPHOCYTIC LEUKEMIA
(10%)
• RICHTER SYNDROME (5%)(AGGRESSIVE LYMPHOMA)
• DEATH OFTEN DUE TO INFECTIONS..
Smudge cells - CLL
CLL One more peripheral blood findings
in CLL is Presence of Smudge cells
( parachute cells). Along with increased number of
normal appearing lymphocytes.
mainly uncontrolled proliferation of myeloid cells.
Males more than females
Splenomegaly – sometimes massive..
**Philadelphia chromosome** Hybrid chromosome with
translocation between the long arm of chr. 9 and long arm of chr.22 . --- t(9:22).
May be present in granulocyte, RBC or platelet precursors in more than 95% of CML..
Absence of these chr. may have worse prognosis.
CML - Clinical Features
15 - 20% of all leukemias; age 25-60Symptoms:
- non-specific- related to hypermetabolism (high cell turnover)- related to splenomegaly
Course:- chronic phase (mean survival, 3-4y)- accelerated phase- blast crisis / myeloid or lymphoid (survival, < 1y)
Therapy: chemotherapy; BMT
CML: Sustained granulocytosis with immature cells; low LAP score; bone marrow pathology;
Philadelphia chromosome LAP – leukocyte alkaline phosphatase