5. Diseases of White Blood Cells - BP Flashcards
What are the 4 main types of leukemias?
- Acute lymphoid
- Acute myeloid
- Chronic lymphoid
- Chronic myeloid
What is the clinical picture in patients with de novo 5q- syndrome?
Older women with refractory macrocytic anemia + normal or elevated platelet counts.
What are the 4 main microscopic findings in MDS?
- Macrocytic erythroid cells with basophilic stippling.
- Hypogranular and hypolobated neutrophils –> Pseudo Pelger-Huet anomaly).
- Micromegakaryocytes
- Normocellular/ hypercellular bone marrow
What is the clinical presentation in patients with MDS?
- Signs and symptoms of cytopenia.
2. 25% have splenomegaly.
What are mainly the microscopic findings in lymphoid leukemias?
High nuclear to cytoplasmic ratio - FEW nucleoli.
What are mainly the microscopic findings in myeloid leukemias?
More prominent cytoplasm - MORE PROMINENT nucleoli.
What are mainly the microscopic findings in acute leukemias?
BLASTS with euchromatin –> SMOOTH nucleus.
What are mainly the microscopic findings in chronic leukemias?
MYELOCYTES (more mature) that do NOT using chromatin –> HETEROCHROMATIN –> GRANULAR nucleus.
Is there a male predominance in CLL?
YES
What are the markers of CLL?
CD19, 20, 23, 5(!)
Mention some associated mutations that we see in CLL.
- del 13q12-14
- Trisomy 12
- del 11q
- del 17p
What is the prognosis in CLLs associated with a mutation?
POOR
Mention some complications of CLL.
- Hypogammaglobulinemia
- 10-15% of patients develop autoimmune hemolytic anemia.
- Immune thrombocytopenia
- 5% –> DLBCL (Richter transformation)
What are the microscopic findings in CLL?
- Cells are CRACKED or GINGERSNAP appearance.
2. Smudge cells during preparation.
What is the clinical presentation of CLL?
Symptoms –> Most ASYMPTOMATIC…some fatigue and weight loss.
Signs –> Normal physical examination…some splenomegaly.
What is the blast crisis seen in CML?
2/3 of CML –> AML
1/3 of CML –> ALL
POOR prognosis.
What is the microscopic morphology of CML?
- Incr. WBC count –> from myelocytes to band and segmented neutrophils.
- BASOPHILS UP!!!
What are the main symptoms of CML?
- 40% of patients –> ASYMPTOMATIC.
- Fatigue + Lethargy + Shortness of breath.
- Weight loss + early satiety due to splenomegaly.
What are the lab findings in CML?
- WBC way up! (median 170.000)
- LAP down.
- High uric acid.
- High lactate dehydrogenase.
What is the clinical course of CML?
Indolent phase 3-5 yrs –> Accelerated phase (fever, weight loss, night sweats etc.) –> Blast crisis.
What percentage of patients with HL have constitutional symptoms?
1/3
What is the MC presenting symptom in HL patients?
Painless cervical lymphadenopathy.
Mention a classic and specific symptom in HL, that is rare.
Pain in the lymph nodes with ingestion of alcohol.
What are the markers for HL nodular sclerosis type?
CD15, 30 + RSCs are negative for EBV.
Where do we find more easily RSCs? In nodular sclerosis or in mixed cellularity HL type?
In mixed cellularity
What are the markers for mixed cellularity HL?
CD15, 30 + RSCs are positive for EBV.
What is the microscopic morphology of lymphocyte predominant HL?
Vaguely nodular –> RSCs variants referred to as L&H, or popcorn cells.
What are the markers in lymphocyte predominant HL?
CD15,30 NEGATIVE + CD20 positive and EMA (epithelial membrane antigen)+.
Mention 2 important points about lymphocyte predominant HL.
- Very benign and often cured with local excision.
2. Often present with cervical or axillary lymphadenopathy.
How do we stage in HL?
Upon the NUMBER of lymph nodes involved (stage I is 1 lymph node) + the LOCATION of lymph nodes in relation to the DIAPHRAGM.
STAGE II –> One or more lymph nodes on the same side of the diaphragm.
STAGE III –> One or more lymph nodes on opposite sides of the diaphragm.
STAGE IV –> Disseminated disease.
Mention an important general point about NHLs.
They are often DISSEMINATED at the time of diagnosis –> aggresive NHLs have a propensity for involving LEPTOmeninges.
What is the typical clinical presentation of NHLs?
- Painless lymphadenopathy (MC neck, inguinal, axillary)
- 20% of patients have constitutional symptoms –> Fever, weight loss, night sweats- “B”symptoms –> common in aggresive NHLs.
What is the main difference between indolent (follicular, SLL) and aggresive (Burkitt, DLBCL) NHLs?
The aggressive are more responsive to chemo, hence they can be cured.
What is the worst NHL?
Mantle cell lymphoma.
What is the OCCASIONAL cytogenetic abnormality in follicular lymphoma?
Translocation involving bcl-6 on 3q27, and not the usual bcl-2 gene on 18.
What are the markers for follicular lymphoma?
CD19, 20, 10, surface Ig, bcl-2.
What is the prognosis for follicular lymphoma patients?
7-9 years –> MOST progress to DLBCL or Burkitt.
Is bone marrow involvement frequent in follicular lymphoma?
Yes –> 85% of cases.
What is the typical clinical presentation of follicular lymphoma?
- Painless lymphadenopathy
- Fever, night sweats, fatigue
- MOST patients (80-90%) present with stage III or IV.
What is the microscopic morphology of SLL?
Proliferation centers - clusters of mitotically active prolymphocytes.
What is the main difference between SLL and CLL?
They are essentially the SAME process.
SLL–> More prominent the involvement of the lymph nodes.
CLL–> More prominent the involvement of the bone marrow and blood.
What is the cytogenetic abnormality in DLBCL?
10-20% have the t(14;18) –> bcl-2
30% have a translocation involving the bcl-6 on 3q27.
What are the markers of DLBCL?
CD19, 20, 79a, surface Ig.
What is the microscopic morphology of DLBCL?
Monotonous sheets of large cells with prominent nucleoli.
Mention 2 important points regarding DLBCL.
- Some cases of DLBCL –> EBV association (in HIV and immunosuppressed patients).
- Primary effusion lymphoma in body cavities –> HHV-8 association.
Besides the classic t(8;14), mention 2 other cytogenetic abnormalities seen in Burkitt lymphoma.
MYC translocated adjacent to Ig light chain gene :
- κ on 2 –> t(2;8)
- λ on 22 –> t(8;22)
What are the markers for Burkitt lymphoma?
CD10, 19, 20, bcl-6, surface Ig.
Mention one important fact about Burkitt lymphoma.
Has the highest turnover rate of ANY human malignancy –> extremely high risk of tumor lysis syndrome.
What is the target group of precursor T cell lymphoblastic leukemia/lymphoma?
Adolescents –> aged 15-20.
What are the markers for precursor T cell lymphoblastic leukemia/lymphoma?
TdT, CD2, 7.
What is the gross morphology of precursor T cell lymphoblastic leukemia/lymphoma?
Usually appears as a mediastinal mass.
What is the microscopic morphology of precursor T cell lymphoblastic leukemia/lymphoma?
Lymphoblasts with irregular nuclear contours, small nucleoli, and scant cytoplasm.
Mention 2 important points regarding precursor T cell lymphoblastic leukemia/lymphoma.
- Propensity involving the bone marrow.
2. Propensity to relapse and involve the LEPTOmeninges.
What are the markers for mantle cell lymphoma?
CD19, 20, 5, Ig, positive - NEGATIVE for CD23 + HIGH levels of cyclin D1.
Mention 2 important points regarding mantle cell lymphoma.
- Survival 3-5 yrs –> NO cure.
2. Can involve GI tract –> producing polyps –> lymphomatoid polyposis.
What is mycosis fungoides/Sezary syndrome?
A chronic T-cell lymphoma with infiltration of the EPIdermis and DERMIS.
Sezary syndrome is a generalized EXFOLIATIVE dermatitis with leukemic spread.
What is the mechanism for the osteolytic lesions in multiple myeloma?
Production of IL-6 by neoplastic cells stimulates osteoclasts through the RANK ligand.
What Ig are produced in multiple myelomas?
60% –> IgG
20-25%–> IgA
15-20% –> λ/κ
What is the main cause of death in multiple myeloma?
Infection (second comes renal failure).
What percentage of multiple myeloma patients do NOT have a detectable M spike, but they do have free light chains in the urine?
20%
What is the microscopic morphology of multiple myeloma?
- > 30% of marrow –> Plasma cells.
- Accumulation of Ig in plasma cells - Russell bodies.
- On peripheral blood smear –> rouleaux formation of RBCs.
Describe briefly the general condition of leukemia.
The neoplastic cells replace the normal hematopoietic population –> patients will have anemia (pallor and fatigue) + thrombocytopenia (bleeding) + leukopenia (infections).
