Patho-Hema Flashcards
Marker for B cells
CD20
LYMPH NODE
Location of mostly B cells
Location of mostly T cells
Cortex
>Lymphoid follicles (primary and secondary) - B cells
>Paracortex - T cells
Media
>Sinuses
Location of naive B cells
Area of B cell activation
Mantle zone
Secondary follicle
Germinal center
Hematologic abnormality in Leukemia
Types of Leukemia
Anemia, Thrombocytopenia, Leukopenia
ALL, AML, ApML
CLL/SLL, CML
Differentiate acute from chronic leukemia in terms of
- Age
- Morphology of cells
- Clinical course
- Response to treatment
Acute
-young, primitive, aggressive, responsive
Chronic
-old, mature, indolent, resistant
Hypercellular marrow with lymphoblasts
Differentiate types in:
age
clinical presentation
mutation
B cell ALL
- children, bone marrow failure
- t(12;21), t(9;22)
T cell ALL
- adolescent, thymus mass
- NOTCH-1
> 20% myeloblast in BM
Aleukemic leukemia
Age
Presentation
Prognosis
AML
Adults
Bone marrow failure
Poor
Cells with Needle-like azurophilic granules
Associated with DIC
Dx:
Mutation:
Tx:
Acute Promyelocytic Leukemia
With faggot cells containing auer rods
Mutation in t(15;17)
Curanle with all-trans retinoic acid
Hepatosplenomegaly
Lymohadenopathy
Hypogammaglobulinemka
Lymph node has lymphocytes with proliferation centers
Peripheral blood smear shows SMUDGE CELLS
CLL >5000 lymphocytes, leakage
SLL <5000, distinct tissue mass
Blasts <10% in peripheral blood
WBC >100000
Thrombocytosis
Accelerated phase: unresponsive to therapy, increasing splenomegaly
Blast crisis: >20%, chloroma
Mutation?
Tx?
CML
t(9;22) Philadelphia gene
With tyrosinase activity
Tx: tyrosine kinase activity inhibitor (Imatinib)
Differentiate leukemoid reaction from leukemia
Leukocyte alkaline phosphatase:
Inc. in leukemoid (reactive AP)
Dec. In CML
Differentiate hodgkin from nonhodgkins lymphoma
HL- single axial nodes (cervical, mediastinal, para aortic)
NHL- multiple, peripheral
- extranodal presentation
- mesenteric ring and waldeyer ring involvement
Large, multiple nuclei or single with multiple lobes, each with owl-eye nucleolus
Reed-Sternberg cells in Hodgkins lymphoma
HL type with reactive B cells? Marker
HL types associated with EBV?
Most common HL type?
HL associated with HIV?
Tx of HL
Lymphocyte predominant, CD20
Lymphocyte depleted, Mixed cellularity
Nodular sclerosis
Lymphocyte depleted
Radiotherapy
Anti CD30
Types of NHL
B cell lymphomas
T and NK cell lymphomas
Most common form of NHL
Most common lymphoma in adults
Most common indolent lymphoma in adults
Fastest growing human tumor
DLBCL
DLBCL
Follicular lymphoma
Burkitt lymphoma
Middle age
Painless, generalized lymphadenopathy
Lymph node shows nodular or diffuse aggregate of lymphoma cells and BMA shows paratrabechular lymphoid aggregates
FOLLICULAR LYMPHOMA
B cell lymphoma
Indolent, incurable
May transform to DLBCL or Burkitt
Present in male, 60yo
Rapidly enlarging nodular mass
Diffuse pattern of growth of large cells with highly anaplastic appearance
Fatal without treatment
DIFFUSE LARGE B CELL LYMPHOMA
B cell lymphoma
CD45 (lymphoid), CK (epithelial), Vimentin (mesenchymal)
Diffuse infiltration if medium sized anaplastic lymphocytes, punctuated sith macrophages ghah phagocytose apoptotic lymphocytes
BURKITT LYMPHOMA
B cell lymphoma
Starry Sky pattern
Endemic: mandibular, kidney, adrenal, gonads; latent infection with EBV
Sporadic: ileoceum, peritoneal
MYC t(8;14) Aggressive, responsive to chemo
Arises in tissues on chronic inflammation like Sjogren syndrome, Hashimoto thyroiditis, H. pylori gastritis
MARGINAL ZONE LYMPHOMA
Memory B cell origin
Infiltration of epidermis and upper dermis T cells with cerebriform nuclei
MYCOSIS FUNGOIDES
CUTANEOUS T CELL LYMPHOMA
Sezary cells
CD4 helper t cells in skin
65yo
With multiple lytic punched out bone lesions, fracture, renal failure
Lymphocytes, plasma cells and plasma ytoid lymphocytes with mast cell hyperplasia
PAS+ cytoplasmic and nuclear inclusions containing Ig
MULTIPLE MYELOMA
Most important plasma cell neoplasm
Russell bodies
Dutcher bodies
Hypercalcemia > fracture, lytic bone lesions
Dec Ig > recurrent bacterial infections
Bence Jones Proteinuria > renal failure
Ringed sideroblasts
Prusian blue
Iron laden mitochondria in erythroblasts
Nuclear budding abnormalities
ERYTHROID
Myelodysplastic Syndrome
Pseudo-Pelget Huet cells
-PMNs with 2 lobes
GRANULOCYTIC
Myelodysplastic syndrome
Pawn ball
-megakaryocytes
MEGAKARYOCYTIC
Myelodysplastic syndrome
Increase in all cell lines, more erythroid lines
Erythromelalgia - throbbing and burning of hands and feet due to thrombotic occlusoon
Hyperuricemia
Thrombosis
Infarcts
Dx?
Tx?
POLYCYTHEMIA VERA
Phlebotomy
JAK2 mutation
Increase in megakaryocytic lines
Giant platelets in peripheral blood
Thrombosis
Erythromelalgia
Hemorrhage
ESSENTIAL THROMBOCYTOSIS
chemotherapy
Extensive deposition of collagen in marrow by non neoplastic fibroblasts
Pronounced hepatosplenomegaly
Hyperuricemia
Increased WBC and platelets
Dacrocytes
PRIMARY MYELOFIBROSIS
Hematopoietic stem cell transplant
Langerhab cells
Birbeck granules
HISTIOCYTOSIS
Thymic hypoplasia
DiGeorge Syndrome
Thymic hyperplasia
Myasthenia gravis
Autosomal dominant
Defects in spectrin, ankyrin, band 3 and band 4.2
Spherical cells without central pallir
Dx?
Type and Site of hemolysis
Diagnostic?
Tx?
HEREDITARY SPHEROCYTOSIS
Intrinsic, Extravascular
Osmotic fragility testing
Splenectomy
X linked recessive
Dec NADPH
Heinz bodies
Bite cells
Dx?
Type and Site of hemolysis
Diagnostic?
Tx?
G6PD DEFICIENCY
Intrinsic, Both
Newborn screening
Aversion to triggers
Autosomal recessive
6th glu to val mutation
Howell jolly bodies
Dx?
Type and Site of hemolysis
Diagnostic?
Tx?
SICKLE CELL ANEMIA
intrinsic, extravascular
Hgb electrophoresis
Hydroxyurea
Alpha thalassemia
No. Of genes affected: 4, 3, 2, 1
Beta thalassemia
Alpha
- presents at birth
- hydrops fetalis, HbH, a thalassemi trait, silent carrier
Beta
- 5-6mos
- major: HbF, minor: HbA a2d2
Intrinsic, extravascular
Nocturnal hemoglobinuria
Thrombosis
PIGA mutations (CD55, CD59, C8)
Dx?
Type and Site of hemolysis
Diagnostic?
Tx?
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
Extrinsic, Extravascular
Flow cytometry
Hematopoietic stem cell transplant
Ecalizumab
Macroangiopathic hemolytic anemia: cardiac valve prosthesis
Microangioathic hemolytic anemia: DIC, TTP HUS, SLE
Extrinsic, intravascular
Vit B12 deficiency Gastrectomy Ileal resection Methotrexate VitB9 deficiency
Megaloblastic anemia
Most common nutritional disorder
Microcytic hypochromic
Prussian blue
IDA
Decreased ferritin
Dec serum fe/tibc
Most common cause of anemia in hospitalized patients
IL6 > Hepcidin > dec iron transfer > storage pool to bone marrow
Decreased transferrin
Inc ferritin
ACD
Pancytopenia
Most common cause: DRUGS
Hypocellular bone marrow
Dry tap
APLASTIC ANEMIA
Suppression of erythroid elements in bone marrow
Autoimmune
Parvovirus B19
Absent erythroblast in bone marrow but still with platelets and granulocytes
PURE RED CELL APLASIA
Antibodies against gpIIb-IIIa and gbIb-IX IgG
Increased megakaryocytes
Congestion, follicular hyperplasia of spleen
ITP
-dec PC, inc BT
Acute
- postviral
- self limited
Chronic
- sle, hiv, cll
- glucocorticoid
- anti cd20 (Rituximab)
HUS triad
-normal PC
TTP pentad
-dec PC, inc Bt
HUS
- Microangiopathic hemolytic anemia
- Thrombocytopenia
- Renal failure
TTP
+
4. Fever
5. Neurologic manifestations
GpIb-IX deficiency
Platelet adhesion problem
GpIIb-IIIa deficiency
Platelet aggregation problem
Bernard Soulier Disease
dec platelet, inc BT
Glanzmann thrombasthenia
Inc BT
Most common inherited bleeding disorder
Autosomal dominant
Inc BT, inc PTT
Von Willebrand Dse
Desmopressjn
Factor VIII and vWF replacement
Most common hereditary syndrome associated with life threatening bleeding
X linked recessive
FVIII and FIX , FXI deficiency (A,B,C)
HEMOPHILIA
inc PTT
Factor replacement
