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Flashcards in Hemepath Deck (25):
1

Acute Lymphoblastic Leukemia/Lymphoma
pre B cell ALL

Tdt +
CD 10, 19,20
Favorable: t(12;21)
Unfavorable: t (9;22)
young (10)

2

Acute Lymphoblastic Leukemia/Lymphoma
pre T cell ALL

Mediastinal Mass(may cause dysphagia or vena cava syndrome)
Associated with Down syndrome
TdT+
CD2-8
Adolescent male, thymic involvement

3

Acute Myelogenous Leukemia

Older (esp>60)
Myeloblasts >20% (don't forget about Myelodysplastic syndrome-nuclear irregularity, nuclear budding, multinucleation, separated nuclear lobes)
MPO +
Alpha naphthyl butyrate esterase
CD 13, 33, 34, 117
Auer rods
Favorable:
t (8;21)
t (15;17)=Acute promyelocytic leukemia--transposition of retinoic receptor gene-tx-all trans retinoic acid-crystal aggregates of MPO-auer rods-->DIC
Inv(16)
NPM
Unfavorable
T (11q23;v)
FLT3

4

Chronic Leukemia:
Chronic lymphocytic leukemia/Small lymphocytic lymphoma (CLL/SLL)

>60y
B-cell
CD 5 (pan t-cell marker), CD 20, CD23
Smudge cells
Hypoglobulinemia
Autoimmune Hemolytic anemia
CLL with increasing lymph node/spleen may develop to Richter syndrome: large B cell lymphoma

5

Chronic Leukemia:
Hairy cell Leukemia

Middle aged man with pancytopenia, splenomegaly of red pulp, and infections
-mature B cells w/ filamentous hair-like projections
-TRAP+
-Dry tap on BM aspiration
-Tx: cladribine (2-CDA)-->inhibits adenosine deaminase
-good prognosis

6

Chronic Leukemia
Adult T cell Leukemia/Lymphoma

tumor of CD4+ T cells due to HTLV-1 infection
-especially Japan
-punched out bone lesions, hypercalcemia, hepatosplenomegaly, LAD
-floret-like lymphocytes
-usually fatal

7

Hodgkin Lymphoma

bimodal age involvement
low grade fever, night sweats, weight loss
Classical Types
1. Lymphocyte rich (best prognosis)
2. Mixed cellularity
3. Lymphocyte depleted
4. Nodular sclerosis
(CD 15, CD 30)
5. Nodular lymphocyte(CD 20, 45)

more RS-->worse prognosis
RS secrete cytokines-->stimulate reactive cells that make up majority of the tumor mass

8

Lymphocyte rich subtype

Reactive small lymphocytes predominate, few mononuclear or classic Reed-Sternberg cells
CD 15, 30

9

Mixed cellularity

Reed-Sternberg cells and variants on a mixed cellular background including eosinophils, plasma cells, T-lymphocytes, histiocytes
CD 15, 30

10

Lymphocyte depleted type

Paucity of lymphocytes and relative abundance of Reed-Sternberg cells
CD 15, 30

11

Nodular sclerosis

fibrous nodular pattern, lacunar cells
CD 15, 30

12

Nodular lymphocyte predominant subtype

nodularity with predominance of mature lymphocytes and popcorn cell or L & H variant of RS cells
CD 20, 45

13

Non-Hodgkin Lymphoma
Diffuse Large B cell Lymphoma
Large Cell

-most common non hodgkin lymphoma in adults
-usually older adults
-clinically aggressive
-Germinal center B cell or activated b cell(worse)
-30% arise from follicular lymphoma t (14:18)

((14;18) or Bcl6) + Myc -->double hit

14

Non-Hodgkin Lymphoma
Burkitt Lymphoma
Medium Cell

t(8;14) -->over expression of c-myc-->increased proliferation

Sporadic: abdomen or pelvis
Endemic: Mandible, Africa, latent EBV

Rapid growth-->cell death-->macrophages "tingible bodies" clean up--->starry sky pattern

15

Non hodgkin Lymphoma
Follicular Lymphoma
Small Cell

40% of adult NHL
CD 20+ B cells

T(14;18) -->bcl2 over amplification-->anti apoptotic

waxing and waning painless lymphadenopathy

Treatment=rituximab *anti-CD20

-may transform to DLCL in 30% to 50% of cases (

16

Non hodgkin Lymphoma
Mantle Cell Lymphoma
Small Cell

4% of NHLs, older males
CD 5, CD20 B cells
t (11;14) -->over amplification of Cyclin D1 -->increased cel proliferation

17

Non hodgkin Lymphoma
Marginal Zone Lymphoma
Small Cell

associated with chronic inflammatory states
-hashimoto's, sjogren's, H pylori
-may regress w treatment (e.g. H pylori_)

18

Non Hodgkin Lymphoma
T-cell NOS

Lymphadenopathy, eosinophilia, pruritus, fever weight loss
-lack of specific histological features (wastebasket diagnosis)
CD3+ with loss of other pan T cell markers and genetic analysis

19

Plasma Cell Neoplasm
Multiple Myeloma

Chronic monoclonal plasma cell proliferation dependent on IL-6
CRAB
hyperCalcemia
Renal insufficiency
Anemia
Bone lytic lesions/Back pain

Increased risk of infection (#1 cause of death)
-Elevated serum protein b/c increased monoclonal IgG or IgA!!!
-Blood smear shows rouleaux formation

Diagnosis: Monoclonal M spike

Frequent cytogenetic abnormalities: FGFR3, Cyclin D1 and Cyclin D3 genes

Median age: 70 years

Bence jones proteinuria causing renal insufficiency
Light chain toxic to tubular epithelium. Amyloidosis of AL type

20

Plasma cell neoplasm
Monoclonal Gammopathy of Undetermined Significance (MGUS)

asymptomatic precursor to multiple myeloma
1-2% go on to develop MM each year

-most common plasma cell dyscrasia (3% persons older than 50 years old)

21

Plasma cell neoplasm
Waldenstrom macroglobulinemia

M spike=IgM
hyperviscosity of blood--> hyperviscosity syndrome
-headaches
-blurred vision or visual loss
-epistaxis
-leg cramps
-confusional episodes
-NO lytic bone lesions

22

Chronic Myelogenous Leukemia

Peak age 45-85
t (9;22)-->bcr-abl
fusion with tyrosine kinase activity
increased neoplastic granulocytic precursors
-Mutation in pluripotent stem cell that can produce myeloid or lymphoid cells

Peripheral blood smear= increase neutrophils, metamyelocytes, basophils

Enlarging spleen suggests transition of ALL or AML

Tx=imatinib (inhibits bcr-abl tyrosine kinase)

Compare to leukemoid rxn
CML: immature and mature cells, white cell to red ratio off, marked splenomegaly

Leukemoid rxn: +leukocyte alkaline phosphatase, normal basophils, no t(9;22)

23

Polycythemia vera

JAK2 point mutation
-low EPO
-elevated hematocrit
-normal PaO2
-increase # of mast cells-->itching after showering

-hyperviscosity of blood-->claudication, ischemic digits, visual disturbances, digital extremity erythromelalgia (burning with discoloration), budd-chiari syndrome(abdominal pain, ascites, liver enlargement) , 70% hypertension

Tx=phlebotomy

24

Essential Thromboyctosis

JAK2, CALR, and MPL point mutations
-proliferation of predominatly megakaryocytic lineage cells
Symptoms: thrombosis/hemorrhage

No propensity to hyperuricemia/gout with ET because no nucleus present in platelets

25

Primary Myelofibrosis

Jak2, CALR, MPL point mutations
Initially hypercellular marrow

Later atypical megakaryocytic hyperplasia-- fibroblast proliferation-->collagen deposition in BM--> necessity of extramedullary hematopoiesis-->dacrocytes (tear drop)

symptoms: severe fatigue, splenomegaly and hepatomegaly-->early satiety and abdominal discomfort, anemia

Tx: ruxolitinib (Jak2 inhibitor)