Hematopoietic Stem Cell Disorders
Multiple Myeloma and Related Disorders
Erythrocyte Disorders
Iron Overload Syndromes, RBC pathology, and Hemolytic Anemias
Important Topics
100
This acquired hematopoietic stem cell disorder is characterized by severely decreased bone marrow and pancytopenia.
What is aplastic anemia?
100
The M protein in plasma cell dyscrasias can be screened with this test
What is SPEP or UPEP
- points for either one
- confirm w/FLC after
100
The differential for microcytic anemia includes one of these two anemias
(points for either 1)
What is IDA or Thalassemia
100
The immunoglobulin class in Cold Agglutinin disease
What is IgM
- Warm weather is Great (IgG= warm agglutinin disease)
100
The only curative treatment for myelodysplastic syndrome
What is allogenic hematopoietic stem cell transplantation
- hypomethylating agents only reduce progression to AML
200
This is one of the many causes of pure red cell aplasia
What is:
200
This disease is characterized by proteinuria, restrictive cardiomyopathy causing diastolic heart failure, hepatosplenomegaly with evidence of portal hypertension, carpal tunnel syndrome, and macroglossia
What is AL Amyloidosis
200
Low MCV + elevated RDW + PBS showing microcytosis + anisopoikilocytocis is pathognomonic for which disease?
IDA
200
Name a disease which may have target cells
HALT for the target
H: HbC (hemoglobin C) disease
A: Asplenia
L: Liver disease
T: Thalassemia
200
This is the translocation causing CML
9:22
- long arms
- will also accept Philadelphia chromosome, BCR-ABL fusion gene (product of the translocation)
300
Clinically significant infections occur around this level of Neutrophil count, classified as severe neutropenia
What is < 500 uL
1000-1500 mild
500-1000 moderate
300
This formation is seen in pt's with MM
Rouleux formation
- also points for "erythrocytes (or RBC's) stacked on one top of another"
300
MCV 72-100 + low serum iron + low TIBC (< 250) + elevated ferritin classifies which class of anemia?
Inflammatory anemia
300
"Hook osteophytes" of particularly the 2nd and 3rd MCP joints, is pathognomonic for this disease
What is hereditary hemochromatosis?
- may also have DIP and PIP involvement
300
Therapy with this class of medications is required for all stages of CML
What are tyrosine kinase inhibitors?
- if you say Imatinib, Dasatinib, and Nilotinib, will accept too
400
This hematologic malignancy is contains a high amount of mature granulocytes (upper left), less mature myelocytes (bottom right), and metamyelocytes (top right). Basophils differentiate this disease from a leukemoid reaction.
What is CML?
400
Name one of the 3 risk factors for progression of MGUS to a clinically significant symptomatic plasma cell dyscrasia
(do NOT need exact values)
400
Microcytic anemia (60-70) + Hemoglobin 10-12 + isolated increase in alpha2 globulin on hemoglobin electrophoresis is the script for this disease
What is beta thalassemia?
400
The treatment for secondary iron overload
What is oral iron chelation therapy
ex: Deferasirox or Deferiprone
- therapeutic phlebotomy is contraindicated since they're anemic
400
Pruritus after a warm shower differentiates this cause of secondary erythrocytosis (> 16.5 g/dL hemoglobin) from other causes
What is polycythemia vera
500
This mutation is found in 97% of all pts with Polycythemia vera, 50% of all patients with essential thrombocytopenia and primary myelofibrosis
What is the Jak2 mutation?
500
This is the CRAB criteria for myeloma-related signs and symptoms
(all 4 required)
Hypercalcemia
Renal failure
Anemia
Bone disease (> 1 lytic lesion on imaging studies)
500
This treatment of paroxysmal nocturnal hemoglobinuria reduces complications and improves quality of life
What is Eculizumab, monoclonal antibody to C5
500
85% to 90% of patients with HH are homozygous for this mutation
- the mutation, not just the gene
What is the C282Y mutation
- in the HFE gene
500
The rod shaped inclusion body known as the Auer rod is seen in which disease?
Acute Myeloid Leukemia
tx: induction therapy w/anthracycline, infusional cytarabine, followed by consolidation therapy for responders. High risk= allogenic hematopoietic stem cells