WBC, Lymph Nodes, Spleen and Thymus Flashcards
Decreased number of circulating leukocytes
MC involves neutrophils (NEUTROPENIA)
Deficiency of lymphocytes (LYMPHOPENIA) - less common; commonly seen in advanced HIV and other diseases
LEUKOPENIA
Clinically significant neutropenia <500/mm3
AGRANULOCYTOSIS
-high susceptibility to infections (Candida and Aspergillus)
-DRUG TOXICITY - MCC
Enlargement of a lymph node as immune response to foreign antigens; histology usually nonspecific; depends on duration of disease and type of offending agent
Reactive Lymphadenitis
7/F with OSA secondary to CHRONIC HYPERTROPHIC TONSILS, GR 3; underwent BILATERAL TONSILLECTOMY and ADENOIDECTOMY
Biopsy of the adenoids: increase in the number of germinal centers (secondary follicles) per unit area
DIAGNOSIS
Follicular Hyperplasia
27/F developed FEVER and LYMPHADENOPATHY.
Serology: heterophil antibodies (+)
Diagnosis: Infectious Mononucleosis
Lymph node biopsy: expansion of the paracortical zones imparting a mottled appearance
Diagnosis
Paracortical Hyperplasia
59/F, known case of breast cancer, underwent SENTINEL LYMPH NODE BIOPSY
Lymph node biopsy: (-) evidence of metastasis; (+) distention of lymphatic sinusoids by histiocytes
Sinusoidal Hyperplasia/Sinus Histiocytes
Neoplastic proliferation of white cells wherein the neoplastic white cells populate the peripheral blood and bone marrow which leads to peripheral blood cytopenias.
Clinical manifestations depend on the cell line/s affected
Leukemia
Neoplastic proliferation of white cells that presents as DISCRETE TISSUE MASSES
Lymphoma
4/F presents w/ PALLOR, RECURRENT URTI and EPISTAXIS
PE: increased liver span and obliterated Traube space
CBC: 35% blast count w/ lymphocytic background
BM: hypercellular marrow w/ diffuse infiltration of blast cells
Flow Cytometry: 89% bone marrow blasts
Acute Lymphoblastic Leukemia (ALL) (precursor B-cell ALL)
Myeloid markers: MPO, CD117, CD33 (-)
Lymphoid markers: Tdt (+)
B-cell lineage markers: CD79a, CD19 (+)
T-cell lineage markers: CD3, CD7 (-)
12/M presents w/ MEDIASTINAL MASS
CT: multiple mass in the abdomen and mediastinum
CBC: 40% blast count w/ lymphocytic background
BM: hypercellular marrow w/ diffuse infiltration of blast cells
Flow Cytometry: 78% bone marrow blasts
Precursor T-cell ALL
Myeloid markers: MPO, CD117, CD33 (-)
Lymphoid markers: Tdt (+)
B-cell lineage markers: CD79a, CD19 (-)
T-cell lineage markers: CD3, CD7 (+)
40/M presents w/ BLEEDING
CBC: thrombocytopenia, 15% blasts count, w/ 40% nucleated WBCs w/ Auer rods obscuring the supposed perinuclear clearing (Golgi zone) of a promyelocyte
Coags: elevated PT/PTT
FISH: (+) t(15;17) PML-RARA
Acute Promyelocyte Leukemia (WHO Class I)
36/M, known case of HODGKIN LYMPHOMA stage IIIB underwent chemotherapy w/ alkylating agents developed recurrent epistaxis
PE: increased liver span and obliterated Traube space
CBC: anemia, thrombocytopenia, leukocytosis (200 000/mm3) w/ 40% blast count
BM: hypercellular marrow w/ diffuse infiltration of blast cells
Flow cytometry: 92% BM blasts
Acute Myeloid Leukemia (AML
Therapy Related AML (WHO Class II)
68/M, w/ MULTIPLE LYMPHADENOPATHY
CBC: 300 000/mm3 w/ 95% lymphocytes
PBS: lymphocytosis w/ SMUDGE CELLS
BM: hypercellular marrow w/ nodular lymphoid aggregates
LN Biopsy: diffuse but vaguely nodular infiltration of small lymphocytes w/ PROLIFERATION CENTERS (pseudofollicular pattern)
DIAGNOSIS
Chronic Lymphocytic Leukemia (CLL)
IHC: CD3 (-), CD20, CD5, CD23(+)
45/F apparently asymptomatic
CBC: 300 000/mm3 w/ neutrophilia, left shift (myelocytes and metamyelocytes) and 2% blast count
LAP scores: LOW
Diagnosis
Chronic Myelogenous Leukemia (CML)
FISH studies: (+) BCR-ABL1 t(9:22)
Hodgkin Lymphoma
Lymph node involvement: SINGLE, AXIAL group of nodes (cervical, mediastinal. para-aortic)
Spread: Contiguous
Mesenteric nodes and Waldeyer ring involvement: RARE
Extranodal presentation: RARE
Non-Hodgkin Lyphoma
Lymph node involvement: MULTIPLE, PERIPHERAL NODES
Spread: Non-contiguous
Mesenteric nodes and Waldeyer ring involvement: COMMON
Extranodal presentation: COMMON
23/F, Filipino w/ ling standing SOLITARY LEFT LATERAL MASS w/ fever and night sweats
PPD (+)
LN Biopsy: mixed infiltrate of inflammatory cells w/ large cells w/ multiple nuclei, each of which has a nucleolus (Owl eye) nuclei; (-) fibrous nodules
Mixed Cellularity HL.
IHC: CD15(+) and CD30 (+)
23/F, Filipino w/ ling standing SOLITARY LEFT LATERAL MASS w/ fever and night sweats
PPD (+)
LN Biopsy: (+) fibrous nodules, (+) scattered cells, (-) necrosis
Nodular Sclerosis HL (Grade 1)
23/F, Filipino w/ ling standing SOLITARY LEFT LATERAL MASS w/ fever and night sweats
PPD (+)
LN Biopsy: SCANt lymphocytic infiltrate w/ predominance o large pleomorphic cells; some of which look like RS cells
Lymphocyte-depleted HL
Processes that distort the marrow architecture, such as deposits of metastatic cancer or granulomatous disorders, can cause the abnormal release of immature precursors into the peripheral blood. This finding is referred to as
Leukoerythroblastosis
MYELOID
erythroid (RBCs)
granulocytic - neutrophils, eosinophils, basophils
monocytic
megakaryocytic - platelets
LYMPHOID
lymphocytes (B and T cells)
plasma cells (from B cells)
natural killer (NK) cells
MCC in children
ALL
Early childhood
BM failure
t(12;21), t(9;22)
B Cell ALL
Adolescents males
thymic masses
lymphadenopathy
splenomegaly
NOTCH-1 (70%)
T Cell ALL
MCC of agranulocytosis
Drug Toxicity
Histologic findings in ALL
LYMPHOBLASTS - scant basophilic cytoplasm and larger nuclei
Hypercellular marrow with LYMPHOBLASTS
PAS (+)
Unique metastatic sites of ALL
CNS
testis
Neoplastic proliferation of myeloid precursor
> 20% myeloblasts in BM
AML
FAB M3 - BEST prognosis
Therapy related - POOR prognosis
Peculiar manifestations of AML
DIC (APML)
Mucocutaneous deposits (AML w/ monocytic differentiation)
Myeloid sarcoma (soft tissue mass)
Acute Promyelocytic Leukemia (APML)
associated with DIC
t (15;17)
AUER RODS - needle like azurophilic granules in cytoplasm
FAGGOT CELLS - w/ numerous Auer rods
MC leukemia of adults in WESTERN world
AUTOIMMUNE
constitutional symptoms
hepatosplenomegaly, lymphadenopathy
immune abnormalities: infection or AIHA or ITP
CLL/SLL
CLL > 5000
SLL <5000
Histologic findings of CLL/SLL
SHEETS OF SMALL LYMPHOCYTES
PROLIFERATION CENTERS - pathognomonic
SMUDGE CELLS - disrupted cells from smearing
Notable sequela of CLL
RICHTER SYNDROME - progression to DLCBL
BCR-ABL t(9;22)
Philadelphia chromosome
splenomegaly (extramedullary hematopoiesis)
ACCELERATED PHASE - worsening anemia or thrombocytopenia with BASOPHILIA
BLAST CRISIS - resembles acute leukemia
CML
Morphology of CML
HYPERCELLULAR MARROW W/ SEA BLUE HISTIOCYTES
WBC >100 000/mm3
LOW leukocyte alkaline phosphatase (LAP)
blast <10%
THROMBOCYTOSIS
3 PHASES OF CML
CHRONIC phase
ACCELERATED phase
*10-19%
*increased WBC count (unresponsive to therapy)
*increased splenomegaly
BLAST crisis
*>20%
*CHLOROMA - solid focus of leukemia outside BM
Danger signs of CML
INCREASED blast count
INCREASED basophil count
DECREASED platelet count
Single AXIAL group of nodes
CONTIGUOUS
painless lymphadenopathy
LN –> spleen –> liver –> BM
fever, night sweats, weight loss
HODGKIN LYMPHOMA
nodular sclerosis - MC!
mixed cellularity
lymphocyte rich
lymphocyte depletion - least common; worst prognosis; PLHIV
nodular lymphocyte predominance - young adult
Fundamental molecular abnormality in HODGKIN LYMPHOMA
NF-kB activation
The most important prognostic variable in Hodgkin Lymphoma
STAGE
Treatment for Hodgkin Lymphoma
Radiotherapy
Chemotherapy
Anti-CD30 (Brentuxumab)
Immune Checkpoint Inhibitors
MULTIPLE PERIPHERAL NODES
NON - CONTIGUOUS
(+) mesenteric nodes and Waldeyer ring involvement
(+) extranodal presentation
NON - HODGKIN LYMPHOMA
NON HODGKIN LYMPHOMA
PERIPHERAL B CELL LYMPHOMAS
SMALL CELL LYMPHOCYTIC PROLIFERATION
Small lymphocytic lymphoma
Mantle cell lymphoma
Follicular lymphoma
Marginal zone lymphoma
NON HODGKIN LYMPHOMA
PERIPHERAL B CELL LYMPHOMAS
AGGRESSIVE B CELL LYMPHOMAS
Diffuse large B cell lymphoma (DLCBL)
Burkitt lymphoma
PERIPHERAL T CELL AND NK CELL LYMPHOMAS
Anaplastic large cell lymphoma
Mycosis fungoides/Sezary syndrome
MC Non Hodgkin Lymphoma
Diffuse large B cell lymphoma (DLCBL)
Associated w/ LATENT EBV infection
HIGH MITOTIC INDEX + NUMEROUS APOPTOTIC CELLS
STARRY SKY PATTERN
royal blue cytoplasm + clear cytoplasmic vacuoles
BURKITT LYMPHOMA
t(8;14)
ONE OF THE FASTEST GROWING HUMAN TUMOR
splenomegaly
pancytopenia and DRY TAP on BM aspiration
monocytopenia
HAIR-LIKE PROJECTIONS
HAIRY CELL LEUKEMIA (HCL)
MC indolent lymphoma of adults
painless
generalized lymphadenopathy
follicular architecture with uniformly sized follicles with thinned mantle zones
CENTROBLASTS AND CENTROCYTES
FOLLICULAR LYMPHOMA
BCL2; t(14;18)
Peripheral B cell lymphomas that arise in sites of CHRONIC INFLAMMATION
MARGINAL ZONE LYMPHOMA
H.pylori - stomach
Sjogren syndrome - salivary gland
Hashimoto thyroiditis - thyroid gland
Most important plasma cell neoplasm
Multiple Myeloma
65-70 years
Clinical features: CRAB
HyperCalcemia (increased secretion of cytokines [eg, IL-1, TNF-a, RANK-L] by malignant plasma cells osteoclast activity)
Renal involvement
Anemia
Bone lytic lesions (“punched out” on X-ray A ) back pain
MULTIPLE MYELOMA
vertebral column
Histologic findings in Multiple Myeloma
plasmacytosis
plasmablasts
bizarre multinucleated giant cells
FLAME CELLS
MOTT CELLS - with multiple grapelike cytoplasmic inclusions
RUSSELL BODIES - globular CYTOPLASMIC inclusions
DUTCHER BODIES - globular NUCLEAR inclusions
Laboratory findings in Multiple Myeloma
ROULEAUX FORMATION
Plasma cell leukemia
HYPERCALCEMIA
MONOCLONAL GAMMOPATHY (IgG)
BENCE JONES PROTEINURIA (Ig LIGHT CHAIN)
Laboratory findings in Multiple Myeloma
ROULEAUX FORMATION
Plasma cell leukemia
HYPERCALCEMIA
MONOCLONAL GAMMOPATHY (IgG)
BENCE JONES PROTEINURIA (Ig LIGHT CHAIN)
MCC of death in multiple myeloma
INFECTIONS
Renal failure