Patho 4 | Hematologic

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Most common hematologic problem seen in advanced HIV

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Leukopenia

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Formula for ANC

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(WBC - segmenters) / 1000

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2 causes of neutropenia

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Inadequate/Ineffective granulopoeisis

Accelerated removal/destruction

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4 drugs which can cause inadequate granulopoeisis

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Dose-related: chemotherapy

Idiosyncratic: PTU, chloramphenicol, Phenylbutazine, Sulfa, Aminopyrine

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2 kinds of marrow in neutropenia

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Hypercellular - functioning marrow

Hypocellular - dry tap on BMA

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Leukocytosis associated with myeloproliferative

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Basophilic

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Leukocytosis associated with viral infectiona

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Viral

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2 morphologic features of leukocytosis

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Toxic granules - azurophilic

Dohle bodies - sky blue cytoplasmic puddles

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Primary lymphatic follicles enlarge and are transformed into germinal centers

Follicular hyperplasia

What condition?

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Lymphadenitis

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Non-tender swelling of lymph node

Common in inguinal and axillary nodes

What condition?

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Chronic lymphadenitis

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3 morphologc changes seen in chronic lymphadenitis

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Follicular hyperplasia

Paracortical hyperplasia

Sinis histiocytosis

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3 cells seen in follicular hyperplasia of chronic lymphadenitis

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Centroblasts - proliferating blast-like B cell

Centrocytes - B cells with cleaved nuclear contours

Tingible-body macrophages - phagocytic macrophages containing nuclear debris

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Trace pathway of WBC maturation

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Naive cell > Mantle cell > Germinal center cells/marginal zone

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3 types of neoplastic WBCs

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Lymphoid neoplasms - Bcell, Tcell, NKcell

Myeloid neoplasms - early hematopoietic progenitors

Histiocytes

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Most common cancer of children

Neoplasts of immature B or T cells

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ALL

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3 differences between myelo and lymphoblasts

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Condensed chromatin

Less conspicuous nucleoli

Smaller cytoplasm

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3 morphologic features of ALL

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Hypercellular marrow with lymphoblasts

MPO neg

CALLA pos

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Leukemia most responsive to chemotherapy

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ALL

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Chromosome profile of ALL

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t(12,21)

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DOC for ALL

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Asparaginase

WOF panceatitis

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Method for delivering chemotherapy drugs to CNS sanctuary sites

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Ommaya reservoir

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What condition?

Leukemia that occurs at 15-39 years of age?

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AML

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3 morphologic features of AML

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Hypercellular marrow with myeloblasts

Auer rods - needle-like, numerous in APML

MPO/CD 34 positive

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Genetic profile of acute promyelocytic leukemia

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t(15,17)

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Coagulation disorder highly associated with APML

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DIC

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Treatment for APML

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All-trans retinoic acid

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Genetic profile of CML

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t(9,22)

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Peak incidence of CML

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4th decade of life

25-60 yrs

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Morphologic profile of CML

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Sea-blue histiocytes

Myeloblasts < 10%

Low leukocyte alk phos

WBC > 100,000

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3 stages of CML

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Chronic

Accelerated

Blast

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WHO criteria for accelerated phase in CML

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10-19% myeloblasts

> 20% basophils

Platelet < 100,000 no Tx

Platelet > 1M

Cytogenetic evolution

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WHO criteria for blast crisis

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> 20% myeloblasts/lymphoblasts

Chloroma - solid focus outside a bone marrow

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DOC for CML

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Imatinib

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Most common leukemia of adults and elderly

(median age of 60yrs)

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CLL

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Complication of CLL

Transformation into a diffuse large B cell lymphoma

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Richter syndrome

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Mature B cell tumor in the elderly

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Hairy cell tumor

* stained using tartrate resistant acid phosphatase

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3 features of leukemoid reactions

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> 50,000 WBC

Elevated NAP/LAP

Elevated CRP

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Diagnostic histologic finding of Hodgkin's lymphoma

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Reed-Sternberg cells

"owl's eye"

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Which lymphoma shows involvement of Waldeyer's ring?

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Non-hodgkin's lymphoma

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5 types of Hodgkin's lymphoma

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Nodular sclerosis

Mixed cellularity

Lymphocyte rich - depleted - predominant

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Hodgkin's with the worst prognosis?

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Lymphocyte depleted

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Hodgkin's associated with EBV

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Lymphocyte depleted

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Most common type of Hodgkin's lymphoma

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Nodular sclerosis

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Name of staging used for Hodgkins

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Ann Arbor

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Most common hematopoietic malignancy

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NHL

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Most common form of indolent NHL

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Follicular

t(14,18)

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2 histologic findings of follicular lymphoma

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Centroblast

Centrocytes

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Most common form of NHL

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Diffuse large B-cell lymphoma

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Lymphoma associated with translocations of c-MYC on chromosome 8

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Burkitt's lymphoma

t(8,14)

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Histologic pattern seen on Burkitt's lymphoma

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Starry sky pattern

Burkitt cells - interspersed macrophages

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Genetic profile of mantle cell lymphoma

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T(11,14)

Lacks cyclin D1

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3 morphologic features of mantle cell lymphadenopathy

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Homogenous popn of small lymphocytes

No centroblasts

No proliferation centers

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Oncogene causing adult T-cell lymphoma

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HTLV 1

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Histologic finding of adult T-cell lymphoma

*cells with multilobulated nuclei

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Clover-leaf or flowe cells

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3 manifestations of mycosis fungoides

*cutaneous T-cell lymphoma

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Mycosis fungoides - chronic proliferative

d'emblee - aggressive nodular eruptive

Sezary syndrome - diffuse erythema and scaling

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2 histologic findings of mycosis fungoides

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Sezary-Lutzner cells

Pautrier microabscesses

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What substance produced by neoplastic plasma cells lead to B-cell proliferations

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IL-6

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Most common and deadly plasma cell neoplasm

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Multiple myeloma

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Synthesized excess light or heavy chains with complete Igs from neoplastic plasma cells are secreted in the urine aa

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Bence-Jones proteins

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Lytic bone lesions seen in multiple myeloma

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Plasmacytoma

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Fiery red cytoplasm seen in MM

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Flame cells

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Multiple grapelike cytoplasmic droplets seen on MM

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Mott cells

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Pink globular cytoplasmic inclusions in MM

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Russell bodies

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M protein causes RBCs in PBS to stick in linear arrays in MM

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Rouleaux

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3 histologic findings of myelodysplastic syndrome

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Ringed sideroblasts

Pseudo-Pelger-Huet

Pawn ball megakaryocytes

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Associated mutation with chronic myelodysplastic disorders

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JAK 2 mutations

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Condition?

Panmyelosis

Polycythemia

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Polycythemia vera

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3 morphologic findings of polycythemia vera

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Hypercellular marrow

Spent phase

Massive hepatosplenomegaly

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Treatment for polycythemia vera

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Phlebotomy

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Diagnosis of exclusion

Marked thrombocytosis

Giant platelets

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Essential thrombocytosis

*activating point mutations in JAK2

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Throbbing/burning of hands and feet due to occlusion of small arterioles

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Erythromelalgia

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Hallmark is the development of obliterative marrow fibrosis

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Primary myelofibrosis

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Tennis racket like granules seen in Langerhans cell histiocytosis

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Birbeck granules

Langerin

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Small yellow-brown, brown or rust colored foci seen in congestive splenomegaly

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Gandy-Gamma nodules

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Average volume of a red cell

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MCV

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Average Hg per red cell (mass)

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MCH

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Average concentration of Hg in a given volume of packed red cells

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MCHC

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3 mechanisms of hemolytic anemias

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Premature destruction of red cells

Elevated EPO

Accumulation of Hg degradation products

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Erythroid precursors seen in the bone marrow in hemolytic anemia

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Normoblasts

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PBS of hemolytic anemias

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Normo

Normo

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Deficient RBC cell membrane proteins in hereditary proteins

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Ankyrin

Band 3

Spectrin

Band 4.2

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Small hyperchromic RBCs lacking central pallor

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Spherocytes

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Etiologic agent in aplastic crisis of hereditary spherocytosis

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Parvovirus B19

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Etiologic agent in hemolytic crisis of hereditary spherocytosis

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EBV mononucleosis

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Small dark nuclesr remnants present in the RBS of asplenic patients

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Howell-Jolly bodies

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Condition where RBCs are unable to protect themselves against oxidative stress

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G6PD

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2 histologic findings of G6PD

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Heinz bodies - membrane bound precipitates

Bite cells - RBC with damaged membranes

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Hallmark of recovery phase in G6PD deficiency

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Reticulocytosis

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Mutation seen in sickle cell anemia

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6th codon of beta-globin

Replacement of glutamate and valine

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Trapping of sickled red cells leading to splenic infarction, fibros and shrinkage

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Autosplenectomy

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Dehydrated RBCs with bull's eye appearance

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Target cells/codocytes

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3 typles of crisis in sickle cell anemia

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Vasooclusive - hypoxix injury and infarction

Sequestration - massive entrapment of sickle cells

Aplastic - Parvoviris B19

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What mechanisms cause the right sided shift of the O2-Hg disassociation curve?

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CO2

Acidosis

Elevated temp

2,3 diphosphoglycerate

Exercise

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DOC for sickle cell anemia

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Hydroxyurea - increases Hbf

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Condition in which inherited mutatiobs decrease synthesis of adult hemoglobin

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Thalassemia

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Thalassemia

Reduced or absent synthesis of alpha-globin chains

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Alpha-thalassemia

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4 types of alpha thalassemia

*depends on how many globin genes are deleted

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Silent carrier - single

Alpha thalassemia - 2, HbA2 levels are normal and low

Hemoglobin H - tetramers of B-globin are formed (HbH), 3, disproportionate tissue hypoxia,

Hydrops fetalis - Hb barts

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Thalassemia

Mutations that diminish synthesis of b-globin chain

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Beta thalassemia

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B-thalassemia type

RBCs completely lack HbA

Major red cell Hg is HbF

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Beta thalassemia major

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B thalassemia

Increase in HbA2 with normal Hbf

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Beta thalassemia minor

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PBS of thalassemia

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Microcytic

Hypochromic

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Marked variation in RBC size

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Anisocytosis

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Marked variation in RBC shape

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Poikilocytosis

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SSx of thalassemia

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Crew cut appearance of skull

Chipmunk facies

Hemosiderosis

Hemochromatosis

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Treatment strategy for thalassemia if not responsive to phlebotomy

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Chelation with deferoxamine

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Intravascular hemolysis due to increased complement-mediated RBC lysis

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PNH

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Deficient proteins in PNH

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DAF

CD55

CD59 - increased risk to undergo lysis, targets membrane-attack complex

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Acute anemia triggered by cold

Mycoplasma pneumonia, infectious mononucleosis

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Cold agglutinin

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Anemia where RBCs are damaged when passing through obstructed or narrow vessel lumen

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Microangiopathic hemolytic anemia

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Associated diseases with microangiopathic hemolytic anemia

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DIC

TTP-HUS

SLE

Malignant hypertension

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2 histologic findings in RBC trauma

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Schistocytes - fragmented RBC, helmet cell

Burr cell/echinocytes - RBC with spikes

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3 histologic findings of megaloblastic anemia

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Macro-ovalocytes

Hypersegmented neutrophils

Hypercellular bone marrow

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Parasitic infection causing megaloblastic anemia

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Diphyllobothrium latum

DOC Praziquantel

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Anemia caused by autoimmune gastritis leading to failure of intrinsic factor production

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Pernicious anemia

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3 morphologic findings of pernicious anemia

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Atrophy of fundic glands

Intestinalization

Atrophic glossitis

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Test for pernicious anemia

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Schilling test

*oral vitB12 and IM vitB12, intrinsic factor

*vitamin B12 deficiency, pernicious anemia, malabsorption

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Most common nutritional disorder in the world

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IDA

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Parasitic infection which results in micro-hypo anemia

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Hookworms (Necator, Ancylostoma)

Albendazole

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Staining for IDA

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Prussian blue stain

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Associated with IDA

Components of Plummer-Vinson syndrome

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Esophageal webs

Micro-hypo

Atrophic glossitis

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MCC of anemia among hospitalized patients

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Anemia of chronic disease

*IL-6 leads to increase in hepcidin

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Syndrome of chronic primary hematopoietic failure and attendant pancytopenoa

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Aplastic anemia

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Drug that causes idiosyncratic aplastic anemia?

Occupational chemical exposure?

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Chloramphenicol

Benzene

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Selective hypoplasia of marrow erythroid elements

Normal granulopoiesis and thrombopoiesis

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Pure red cell aplasia

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Etiology of ITP

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Anti-platelet antibodies

Directed against glycoproteins IIb-IIIa

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Most feared complication of ITP

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Intracranial bleeding

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Treatment for ITP

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Splenectomy

IVIg, anti-CD20 antibody (Rituximab)

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Pentad of TTP

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Fever

Thrombocytopenia

Microangiopathic hemolytic anemia

Transient neurologic deficits

Renal failure

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Triad of hemolytic uremic syndrome

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Microangiopathic hemolytic anemia

Thrombocytopenia

Renal failure

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Causative agent of HUS

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EHEC

O157:H7

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Platelet dysfunction

Decreased gp IB for defective platelet adhesion

Decreased platelet count

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Bernard-Soulier

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Platelet dysfunction

Decreased gp IIb-IIIa leads to defective platelet aggregation

Normal platelet

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Glanzmann's thrombasthenia

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Most common inherited bleeding disorder

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Von Willebrand disease

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Treatment for Von Willebrand

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Desmopressin

Concentrate contaning factor VIII and vWF

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Most common hereditary disease with life-threatening bleeding

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Hemophilia A

*factor VIII deficiency

+ deep bleeding only

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Christmas disease

Factor 9 deficiency

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Hemophilia B

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Acute, subacute, chronic thrombohemorrhagic disorder

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DIC

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Fibrin thrombi lead to massive adrenal hemorrhage

Condition?

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Waterhouse-Friderichsen syndrome

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Postpartum pituitary necrosis

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Sheehan syndrome

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Widespread microthrombi in placenta

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Toxemia of pregnancy

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Diagnostics for DIC

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Fibrinogen

Platelet count

PT/PTT

Fibrin degradation

D-dimer

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Platelet dysfunction comparison table

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Site of hemolysis

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